APPENDIX D.   MAJOR ACTIONS TAKEN UNDER THE NIH GUIDELINES

 

As noted in the subsections of Section IV-C-1-b-(1), the Director, NIH, may take certain actions with regard to the NIH Guidelines after the issues have been considered by the RAC.  Some of the actions taken to date include the following:

 

Appendix D-1.  Permission is granted to clone foot and mouth disease virus in the EK1 host-vector system consisting of E. coli K-12 and the vector pBR322, all work to be done at the Plum Island Animal Disease Center.

 

Appendix D-2.  Certain specified clones derived from segments of the foot and mouth disease virus may be transferred from Plum Island Animal Disease Center to the facilities of Genentech, Inc., of South San Francisco, California.  Further development of the clones at Genentech, Inc., has been approved under BL1 + EK1 conditions.

 

Appendix D-3.  The Rd strain of Hemophilus influenzae can be used as a host for the propagation of the cloned Tn 10 tet R gene derived from E. coli K-12 employing the non-conjugative Hemophilus plasmid, pRSF0885, under BL1 conditions.

 

Appendix D-4.  Permission is granted to clone certain subgenomic segments of foot and mouth disease virus in HV1 Bacillus subtilis and Saccharomyces cerevisiae host-vector systems under BL1 conditions at Genentech, Inc., South San Francisco, California.

 

Appendix D-5.  Permission is granted to Dr. Ronald Davis of Stanford University to field test corn plants modified by recombinant DNA techniques under specified containment conditions.

 

Appendix D-6.  Permission is granted to clone in E. coli K-12 under BL1 physical containment conditions subgenomic segments of rift valley fever virus subject to conditions which have been set forth by the RAC.

 

Appendix D-7.  Attenuated laboratory strains of Salmonella typhimurium may be used under BL1 physical containment conditions to screen for the Saccharomyces cerevisiae pseudouridine synthetase gene.  The plasmid YEp13 will be employed as the vector.

 

Appendix D-8.  Permission is granted to transfer certain clones of subgenomic segments of foot and mouth disease virus from Plum Island Animal Disease Center to the laboratories of Molecular Genetics, Inc., Minnetonka, Minnesota, and to work with these clones under BL1 containment conditions.  Approval is contingent upon review of data on infectivity testing of the clones by a working group of the RAC.

 

Appendix D-9.  Permission is granted to Dr. John Sanford of Cornell University to field test tomato and tobacco plants transformed with bacterial (E.coli K-12) and yeast DNA using pollen as a vector.

 

Appendix D-10.  Permission is granted to Drs. Steven Lindow and Nickolas Panopoulos of the University of California, Berkeley, to release under specified conditions Pseudomonas syringae, pathovars (pv.) syringae, and Erwinia herbicola carrying in vitro generated deletions of all or part of the genes involved in ice nucleation.

 

Appendix D-11.  Agracetus of Middleton, Wisconsin, may field test under specified conditions disease resistant tobacco plants prepared by recombinant DNA techniques.

 

Appendix D-12.  Eli Lilly and Company of Indianapolis, Indiana, may conduct large-scale experiments and production involving Cephalosporium acremonium strain LU4-79-6 under less than Biosafety Level 1 - Large Scale (BL1-LS) conditions.

 

Appendix D-13.  Drs. W. French Anderson, R. Michael Blaese, and Steven Rosenberg of the NIH, Bethesda, Maryland, can conduct experiments in which a bacterial gene coding for neomycin phosphotransferase will be inserted into a portion of the tumor infiltrating lymphocytes (TIL) of cancer patients using a retroviral vector, N2.  The marked TIL then will be combined with unmarked TIL, and reinfused into the patients.  This experiment is an addition to an ongoing adoptive immunotherapy protocol in which TIL are isolated from a patient's tumor, grown in culture in the presence of interleukin-2, and reinfused into the patient.  The marker gene will be used to detect TIL at various time intervals following reinfusion. 

 

Approval is based on the following four stipulations:  (I) there will be no limitation of the number of patients in the continuing trial; (ii) the patients selected will have a life expectancy of about 90 days; (iii) the patients give fully informed consent to participate in the trial; and (iv) the investigators will provide additional data before inserting a gene for therapeutic purposes. (Protocol #8810-001)

 

Appendix D-14.  U.S. Army Medical Research Institute of Infectious Diseases (USAMRIID) may conduct certain experiments involving products of a yellow fever virus originating from the 17-D yellow fever clone at the Biosafety Level 3 containment level using HEPA filters and vaccination of laboratory personnel.

 

In addition, USAMRIID may conduct certain experiments involving vaccine studies of Venezuelan equine encephalitis virus at the Biosafety Level 3 containment level using HEPA filters and vaccination of laboratory personnel.

 

Appendix D-15.  Drs. R. Michael Blaese and W. French Anderson of the NIH, Bethesda, Maryland, can conduct experiments in which a gene coding for adenosine deaminase (ADA) will be inserted into T lymphocytes of patients with severe combined immunodeficiency disease, using a retroviral vector, LASN.  Following insertion of the gene, these T lymphocytes will be reinfused into the patients.  The patients will then be followed for evidence of clinical improvement in the disease state, and measurement of multiple parameters of immune function by laboratory testing.

 

Approval is based on the following two stipulations:  (I) that intraperitoneal administration of transduced T lymphocytes not be used before clearance by the Chair of the Recombinant DNA Advisory Committee; and (ii) that the number of research patients be limited to 10 at this time.

 

In addition to the conditions outlined in the initial approval, patients may be given a supplement of a CD-34+-enriched peripheral blood lymphocytes (PBL) which have been placed in culture conditions that favor progenitor cell growth.  This enriched population of cells will be transduced with the retroviral vector, G1NaSvAd.  G1NaSvAd is similar to LASN, yet distinguishable by PCR.  LASN has been used to transduce peripheral blood T lymphocytes with the ADA gene.  Lymphocytes and myeloid cells will be isolated from patients over time and assayed for the presence of the LASN or G1NaSvAd vectors.  The primary objectives of this protocol are to transduce CD 34+ peripheral blood cells with the adenosine deaminase gene, administer these cells to patients, and determine if such cells can differentiate into lymphoid and myeloid cells in vivo.  There is a potential for benefit to the patients in that these hematopoietic progenitor cells may survive longer, and divide to yield a broader range of gene-corrected cells. (Protocol #9007-002)

 

Appendix D-16.  Dr. Steven A. Rosenberg of the National Institutes of Health, Bethesda, Maryland, can conduct experiments on patients with advanced melanoma who have failed all effective therapy.  These patients will be treated with escalating doses of autologous tumor infiltrating lymphocytes (TIL) transduced with a gene coding for tumor necrosis factor (TNF).  Escalating numbers of transduced TIL will be administered at three weekly intervals along with the administration of interleukin-2 (IL-2).  The objective is to evaluate the toxicity and possible therapeutic efficacy of the administration of tumor infiltrating lymphocytes (TIL) transduced with the gene coding for TNF. (Protocol #9007-003)

 

Appendix D-17.  Dr. Malcolm K. Brenner of St. Jude Children's Research Hospital of Memphis, Tennessee, can conduct experiments on patients with acute myelogenous leukemia (AML).  Using the LNL6 retroviral vector, the autologous bone marrow cells will be transduced with the gene coding for neomycin resistance.  The purpose of this gene marking experiment is to determine whether the source of relapse after autologous bone marrow transplantation for acute myelogenous leukemia is residual malignant cells in the harvested marrow or reoccurrence of tumor in the patient.  Determining the source of relapse should indicate whether or not purging of the bone marrow is a necessary procedure. (Protocol #9102-004)

 

Appendix D-18.  Dr. Malcolm K. Brenner of St. Jude Children's Research Hospital of Memphis, Tennessee, can conduct experiments on pediatric patients with Stage D (disseminated) neuroblastoma who are being treated with high-dose carboplatin and etoposide in either phase I/II or phase II trials.  All the patients in these studies will be subjected to bone marrow transplantation since it will allow them to be exposed to chemoradiation that would be lethal were it not for the availability of stored autologous marrow for rescue.  The bone marrow cells of these patients will be transduced with the gene coding for neomycin resistance using the LNL6 vector.  The purpose of this gene marking study is to determine whether the source of relapse after autologous bone marrow transplantation is residual malignant cells in the harvested marrow or residual disease in the patient.  Secondly, it is hoped to determine the contribution of marrow autographs to autologous reconstitution. (Protocol #9105-005/9105-006)

 

Appendix D-19.  Dr. Albert B. Deisseroth of the MD Anderson Cancer Center of Houston, Texas, can conduct experiments on patients with chronic myelogenous leukemia who have been reinduced into a second chronic phase or blast cells.  The patients in these studies will receive autologous bone marrow transplantation.  Using the LNL6 vector, the bone marrow cells will be transduced with the gene coding for neomycin resistance.  The purpose of these marking studies is to determine if the origin of relapse arises from residual leukemic cells in the patients or from viable leukemic cells remaining in the bone marrow used for autologous transplantation. (Protocol #9105-007)

 

Appendix D-20.  Drs. Fred D. Ledley and Savio L. C. Woo of Baylor College of Medicine of Houston, Texas, can conduct experiments on pediatric patients with acute hepatic failure who are identified as candidates for hepatocellular transplantation.  Using the LNL6 vector, the hepatocytes will be transduced with the gene coding for neomycin resistance.  The purpose of using a genetic marker is to demonstrate the pattern of engraftment of transplanted hepatocytes and to help determine the success or failure of engraftment. (Protocol #9105-008)

 

Appendix D-21.  Dr. Steven A. Rosenberg of the National Institutes of Health, Bethesda, Maryland, can conduct experiments on patients with advanced melanoma, renal cell cancer, and colon carcinoma who have failed all effective therapy.  In an attempt to increase these patients' immune responses to the tumor, the tumor necrosis factor gene or the interleukin-2 gene will be introduced into a tumor cell line established from the patient.  These gene-modified autologous tumor cells will then be injected into the thigh of the patient.  To further utilize the immune system of the patient to fight the tumor, stimulated lymphocytes will be cultured from either the draining regional lymph nodes or the injected tumor itself.  The patients will be evaluated for antitumor effects engendered by the injection of the gene modified tumor cells themselves as well as after the infusion of the cultured lymphocytes. (Protocol #9110-010/9110-011)

 

Appendix D-22.  Dr. James M. Wilson of the University of Michigan Medical Center of Ann Arbor, Michigan, can conduct experiments on three patients with the homozygous form of familial hypercholesterolemia.  Both children and adults will be eligible for this therapy.  In an attempt to correct the basic genetic defect in this disease, the gene coding for the low-density lipoprotein (LDL) receptor will be introduced into liver cells taken from the patient.  The gene-corrected hepatocytes will then be infused into the portal circulation of the patient through an indwelling catheter.  The patients will be evaluated for engraftment of the these treated hepatocytes through a series of metabolic studies; three months after gene therapy, a liver biopsy will be taken and analyzed for the presence of recombinant derived RNA and DNA to document the presence of the gene coding for the normal LDL receptor. (Protocol #9110-012)

 

Appendix D-23.  Dr. Michael T. Lotze of the University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, can conduct experiments on 20 patients with metastatic melanoma who have failed conventional therapy.  A gene transfer experiment will be performed, transducing the patients' tumor infiltrating lymphocytes (TILs) with the gene for neomycin resistance.  Through the use of this gene marking technique, it is proposed to determine how long TIL cells can be detected in vivo in the peripheral blood of the patients, and how the administration of interleukin-2 and interleukin-4 affects localization and survival of TIL cells in tumor sites. (Protocol #9105-009)

 

Appendix D-24.  Dr. Gary J. Nabel of the University of Michigan Medical School, Ann Arbor, Michigan, can conduct gene therapy experiments on twelve patients with melanoma or adenocarcinoma.  Patient population will be limited to adults over the age of 18 and female patients must be postmenopausal or have undergone tubal ligation or orchiectomy.  The patient's immune response will be stimulated by the introduction of a gene encoding for a Class I MHC protein, HLA-B7, in order to enhance tumor regression.  DNA/liposome-mediated transfection techniques will be used to directly transfer this foreign gene into tumor cells.  HLA-B7 expression will be confirmed in vivo, and the immune response stimulated by the expression of this antigen will be characterized.  These experiments will be analyzed for their efficacy in treating cancer. (Protocol #9202-013)

 

Appendix D-25.  Kenneth Cornetta of Indiana University, Indianapolis, Indiana, can conduct gene transfer experiments on up to 10 patients with acute myelogenous leukemia (AML) and up to 10 patients with acute lymphocytic leukemia (ALL).  The patient population will be limited to persons between 18 and 65 years of age.  Using the LNL-6 vector, autologous bone marrow cells will be marked with the neomycin resistance gene.  Gene marked and untreated bone marrow cells will be reinfused at the time of bone marrow transplantation.  Patients will then be monitored for evidence of the neomycin resistance gene in peripheral blood and bone marrow cells in order to determine whether relapse of their disease is a result of residual malignant cells remaining in the harvested marrow or inadequate ablation of the tumor cells by chemotherapeutic agents.  Determining the source of relapse may indicate whether or not purging of the bone marrow is a necessary procedure for these leukemia patients.  Further studies will be performed in order to determine the percentage of leukemic cells that contain the LNL-6 vector and the clonality of the marked cells. (Protocol #9202-014)

 

Appendix D-26.  Dr. James S. Economou of the University of California, Los Angeles, can conduct gene transfer experiments on 20 patients with metastatic melanoma and 20 patients with renal cell carcinoma.  These patients will be treated with various combinations of tumor-infiltrating lymphocytes and peripheral blood leukocytes, including CD8 and CD4 subsets of both types of cells.  These effector cell populations will be given in combination with interleukin-2 (IL-2) in the melanoma patients and IL-2 plus alpha interferon in the renal cell carcinoma patients.  The effector cells will be transduced with the neomycin resistance gene using either the LNL6 or G1N retroviral vectors.  This "genetic marking" of the tumor-infiltrating lymphocytes and peripheral blood lymphocytes is designed to answer questions about the trafficking of these cells, their localization to tumors, and their in vivo life span. (Protocol #9202-015)

 

Appendix D-27.  Drs. Philip Greenberg and Stanley R. Riddell of the Fred Hutchinson Cancer Research Center, Seattle, Washington, may conduct gene transfer experiments on 15 human immunodeficiency virus (HIV) seropositive patients (18-45 years old) undergoing allogeneic bone marrow transplantation for non-Hodgkin's lymphoma and 15 HIV-seropositive patients (18-50 years old) who do not have acquired immunodeficiency syndrome (AIDS)-related lymphoma and who are not undergoing bone marrow transplantation to evaluate the safety and efficacy of HIV-specific cytotoxic T lymphocyte (CTL) therapy.  CTL will be transduced with a retroviral vector (HyTK) encoding a gene that is a fusion product of the hygromycin phosphotransferase gene (HPH) and the herpes simplex virus thymidine kinase (HSV-TK) gene.  This vector will deliver both a marker gene and an ablatable gene in these T cell clones in the event that patients develop side effects as a consequence of CTL therapy.  Data will be correlated over time, looking at multiple parameters of HIV disease activity.  The objectives of these studies include evaluating the safety and toxicity of CTL therapy, determining the duration of in vivo survival of HIV-specific CTL clones, and determining if ganciclovir therapy can eradicate genetically modified, adoptively transferred CTL cells. (Protocol #9202-017)

 

Appendix D-28.  Dr. Malcolm Brenner of St. Jude Children's Research Hospital, Memphis, Tennessee, can conduct gene therapy experiments on twelve patients with relapsed/refractory neuroblastoma who have relapsed after receiving autologous bone marrow transplant.  In an attempt to stimulate the patient's immune response, the gene coding for Interleukin-2 (IL-2) will be used to transduce tumor cells, and these gene-modified cells will be injected subcutaneously in a Phase 1 dose escalation trial.  Patients will be evaluated for an anti-tumor response. (Protocol #9206-018)

 

Appendix D-29.  Drs. Edward Oldfield, Kenneth Culver, Zvi Ram, and R. Michael Blaese of the National Institutes of Health, Bethesda, Maryland, can conduct gene therapy experiments on ten patients with primary malignant brain tumors and ten patients with lung cancer, breast cancer, malignant melanoma, or renal cell carcinoma who have brain metastases.  The patient population will be limited to adults over the age of 18.

 

Patients will be divided into two groups based on the surgical accessibility of their lesions.  Both surgically accessible and surgically inaccessible lesions will receive intra-tumoral injections of the retroviral Herpes simplex thymidine kinase (HS-tk) vector-producer cell line, G1TkSvNa, using a guided stereotaxic approach.  Surgically accessible lesions will be excised seven days after stereotaxic injection, and the tumor bed will be infiltrated with the HS-tk producer cells.  The removed tumor will be evaluated for the efficiency of transduction.  Ganciclovir (GCV) will be administered beginning on the fifth postoperative day.  In the case of surgically inaccessible lesions, the patients will receive intravenous therapy with GCV seven days after receiving the intra-tumoral injections of the retroviral HS-tk vector-producer cells. (Protocol #9206-019)

 

Appendix D-30.  Dr. Albert D. Deisseroth of MD Anderson Cancer Center, Houston, Texas, can conduct gene transfer experiments on ten patients who have developed blast crisis or accelerated phase chronic myelogenous leukemia (CML).  The retroviral vectors G1N and LNL6 which code for neomycin resistance will be used to transduce autologous peripheral blood and bone marrow cells that have been removed and stored at the time of cytogenetic remission or re-induction of chronic phase in Philadelphia chromosome positive CML patients.  Following reinduction of the chronic phase of CML and preparative chemotherapy, patients will be infused with the transduced autologous cells. 

 

This protocol is designed to determine the cause of relapse of CML.  If polyclonal CML neomycin marked blastic cells appear at the time of relapse, their presence will indicate that relapse arises from the leukemic CML blast cells present in the autologous cells infused following chemotherapy.  If residual systemic disease contributes to relapse, the neomycin resistance gene will not be detected in the CML leukemic blasts at the time of relapse.

 

This study will compare the relative contributions of the peripheral blood and bone marrow to generate hematopoietic recovery after bone marrow transplantation and evaluate purging and selection of peripheral blood or bone marrow as a source of stem cells for transplant.  The percentage of neomycin resistant CML cells which are leukemic will be determined by PCR analysis and detection of bcr-abl mRNA. (Protocol #9206-020)

 

Appendix D-31.  Dr. Cynthia Dunbar of the National Institutes of Health, Bethesda, Maryland, can conduct gene transfer experiments on up to 48 patients with multiple myeloma, breast cancer, or chronic myelogenous leukemia.  The retroviral vectors G1N and LNL6 will be used to transfer the neomycin resistance marker gene into autologous bone marrow and peripheral blood stem cells in the presence of growth factors to examine hematopoietic reconstitution after bone marrow transplantation.  The efficiency of transduction of both short and long term autologous bone marrow reconstituting cells will be examined.

 

Autologous bone marrow and CD34+ peripheral blood stem cells will be enriched prior to transduction.  Myeloma and CML patients will receive both autologous bone marrow and peripheral blood stem cell transplantation.  These separate populations will be marked with both the G1N and LNL6 retroviral vectors.  If short and long term marking experiments are successful, important information may be obtained regarding the biology of autologous reconstitution, the feasibility of retroviral gene transfer into hematopoietic cells, and the contribution of viable tumor cells within the autograft to disease relapse. (Protocol #9206-023/9206-024/9206-025)

 

Appendix D-32.  Dr. Bernd Gansbacher of the Memorial Sloan-Kettering Cancer Center, New York, New York, can conduct gene therapy experiments on twelve patients over 18 years of age with metastatic melanoma who are HLA-A2 positive and who have failed conventional therapy.  This is a phase I study to examine whether allogeneic HLA-A2 matched melanoma cells expressing recombinant human Interleukin-2 (IL-2) can be injected subcutaneously and used to create a potent tumor specific immune response without producing toxicity.  By allowing the tumor cells to present the MHC Class I molecule as well as the secreted IL-2, a clonal expansion of tumor specific effector cells is expected.  These effector populations may access residual tumor at distant sites via the systemic circulation. (Protocol #9206-021)

 

Appendix D-33.  Dr. Bernd Gansbacher of the Memorial Sloan-Kettering Cancer Center, New York, New York, can conduct gene therapy experiments on twelve patients over 18 years of age with renal cell carcinoma who are HLA-A2 positive and who have failed conventional therapy.  This Phase I study will examine whether allogeneic HLA-A2 matched renal cell carcinoma cells expressing recombinant human Interleukin-2 (IL-2) can be injected subcutaneously and used to create a potent tumor specific immune response without producing toxicity.  By allowing the tumor cells to present the MHC Class I molecule as well as the secreted IL-2, a clonal expansion of tumor specific effector cells is expected.  These effector populations may access residual tumor at distant sites via the systemic circulation. (Protocol #9206-022)

 

Appendix D-34.  Dr. Michael T. Lotze, University of Pittsburgh, Pittsburgh, Pennsylvania, can conduct experiments on twenty patients with metastatic, and/or unresectable, locally advanced melanoma, renal cell carcinoma, breast cancer, or colon cancer who have failed standard therapy.  Patients will receive multiple subcutaneous injections of autologous tumor cells combined with an autologous fibroblast cell line that has been transduced in vitro with the gene coding for Interleukin-4 (IL-4) to augment the in vivo antitumor effect.  Patients will be monitored for antitumor effect by PCR analysis and multiple biopsy of the injection site. (Protocol #9209-033)

 

Appendix D-35.  Dr. Friedrich G. Schuening, Fred Hutchinson Cancer Research Center, Seattle, Washington, can conduct human gene transfer experiments on patients 18 years of age with breast cancer, Hodgkin's disease, or non-Hodgkin's lymphoma.  A total of 10 patients per year will be enrolled in the studies over a period of four years.  Patients will undergo autologous bone marrow transplantation with a selected population of Interleukin-3 (IL-3) or granulocyte colony-stimulating factor (G-CSF) stimulated CD34(+) peripheral blood repopulating cells (PBRC) that have been transduced with the gene coding for neomycin resistance (neoR) using the retroviral vector, LN.  Patients will be continuously monitored for neoR to determine the relative contribution of autologous PBRCs to long-term hematopoietic reconstitution.  Demonstration of long-term contribution of autologous PBRC to hematopoiesis will enable the use of PBRC alone for autologous transplants and suggest the use of PBRC as long-term carriers of therapeutically relevant genes. (Protocol #9209-027/9209-028)

 

Appendix D-36.  Dr. Friedrich G. Schuening, Fred Hutchinson Cancer Research Center, Seattle, Washington, can conduct human gene transfer experiments on patients 18 years of age with breast cancer, Hodgkin's disease, or non-Hodgkin's lymphoma.  A total of 5 patients per year will be enrolled in the study over a period of four years.  Patients will undergo allogeneic bone marrow transplant with granulocyte colony-stimulating factor (G-CSF) stimulated CD34(+) PBRC harvested from an identical twin that have been transduced with neoR using the retroviral vector, LN.  Patients will be continuously monitored for neoR to determine the relative contribution of G-CSF stimulated allogeneic PBRCs to long-term bone marrow engraftment.   Demonstration of long-term contribution of allogeneic PBRC to hematopoiesis will enable the use of PBRC alone for allogeneic transplants and suggest the use of PBRC as long-term carriers of therapeutically relevant genes. (Protocol #9209-029)

 

Appendix D-37.  Dr. Malcolm K. Brenner of St. Jude Children's Hospital, Memphis, Tennessee, and Dr. Bonnie J. Mills of Baxter Healthcare Corporation, Santa Ana, California, can conduct a multicenter uncontrolled human gene transfer experiment on 12 patients 21 years of age with Stage D Neuroblastoma in first or second marrow remission.  Autologous bone marrow cells will be separated into two fractions, purged and unpurged.  Each fraction will be transduced with the neoR gene by either LNL6 or G1Na.  Patients will be monitored by the polymerase chain reaction (PCR) for the presence of neoR.  The protocol is designed to evaluate the safety and efficacy of the Neuroblastoma Bone Marrow Purging System following high dose chemotherapy. (Protocol #9209-032)

 

Appendix D-38.  Drs. Carolyn Keierleber and Ann Progulske-Fox of the University of Florida, Gainesville, Florida, can conduct experiments involving the introduction of a gene coding for tetracycline resistance into Porphyromonas gingivalis at a physical containment level of Biosafety Level-2 (BL-2).

 

Appendix D-39.  Dr. Scott M. Freeman of Tulane University Medical Center, New Orleans, Louisiana, can conduct experiments on patients with epithelial ovarian carcinoma who have clinical evidence of recurrent, progressive, or residual disease who have no other therapy available to prolong survival.  Patients will be injected intraperitoneally with the irradiated PA-1 ovarian carcinoma cell line which has been transduced with the herpes simplex thymidine kinase (HSV-TK) gene.  The patients will then receive ganciclovir therapy.  Previous, data indicates that HSV-TK+ tumor cells exhibit a killing effect on HSV-TK- cells when exposed to ganciclovir therapy.  Patients will be evaluated for safety and side effects of this treatment. (Protocol #9206-016)

 

Appendix D-40.  Dr. Michael J. Welsh, Howard Hughes Medical Institute Research Laboratories, University of Iowa College of Medicine, Iowa City, Iowa, may conduct experiments on 3 cystic fibrosis (CF) patients 18 years of age with mild to moderate disease.  This Phase I study will determine the:  (1) in vivo safety and efficacy of the administration of the replication-deficient type 2 adenovirus vector, Ad2/CFTR-1, to the nasal epithelium; (2) efficacy in correcting the CF chloride transport defect in vivo; and (3) effect of adenovirus vector dosage on safety and efficacy. (Protocol #9212-036)

 

Appendix D-41.  Dr. Ronald G. Crystal, National Institutes of Health, Bethesda, Maryland, may conduct experiments on 10 cystic fibrosis (CF) patients 21 years of age.  Patients will receive an initial administration of the replication-deficient type 5 adenovirus vector, AdCFTR, to their left nares.  If no toxicity is observed from intranasal administration, patients will receive a single administration of AdCFTR to the respiratory epithelium of their left large bronchi.  Five groups of patients (2 patients per group) will be studied based on increased dosage administration of AdCFTR.  This study will determine the:  (1) in vivo safety and efficacy of the administration of AdCFTR into the respiratory epithelium; (2) efficacy of the correction of the biologic abnormalities of CF in the respiratory epithelium;  (3) duration of the biologic correction; (4) efficacy of the correction of the abnormal electrical potential difference of the airway epithelial sheet; (5) clinical parameters relevant to the disease process; and (6) if humoral immunity develops against AdCFTR sufficient to prevent repeat administration. (Protocol #9212-034)

 

Appendix D-42.  Dr. Kenneth Culver, Iowa Methodist Medical Center, Des Moines, Iowa, and Dr. John Van Gilder, University of Iowa, Iowa City, Iowa, may conduct experiments on 15 patients 18 years of age with recurrent malignant primary brain tumors or lung, melanoma, renal cell carcinoma, or breast carcinoma brain metastases who have failed standard therapy for their disease.  Patient eligibility will be limited to those patients who have measurable residual tumor immediately following the post-operative procedure as demonstrated by imaging studies.  The number of patients treated will be equally divided between the Iowa Methodist Medical Center and the University of Iowa.  If a positive response is observed in any of the first 15 patients, the investigators may submit a request to treat an additional 15 patients.

 

Following surgical debulking, patients will receive a maximum of 3 intralesional injections of the G1TkSvNa vector- producing cell line (VPC) to induce regression of residual tumor cells by ganciclovir (GCV) therapy.  Patients who demonstrate stable disease for a minimum of 6 months following this treatment will be eligible for additional VPC injections and subsequent GCV therapy. (Protocol #9303-037)

 

Appendix D-43.  Drs. Malcolm Brenner, Robert Krance, Helen E. Heslop, Victor Santana, and James Ihle, St. Jude Children's Research Hospital, Memphis, Tennessee, may conduct experiments on 35 patients 1 year and 21 years of age at the time of initial diagnosis of acute myelogenous leukemia (AML).  The investigators will use the two retroviral vectors, LNL6 and G1Na, to determine the efficacy of the bone marrow purging techniques:  4-hydroxyperoxicyclophosphamide and interleukin-2 (IL-2) activation of endogenous cytotoxic effector cells, in preventing relapse from the reinfusion of autologous bone marrow cells. (Protocol #9303-039)

 

Appendix D-44.  Drs. Helen E. Heslop, Malcolm Brenner, and Cliona Rooney, St Jude Children's Research Hospital, Memphis, Tennessee, may conduct experiments of 35 patients 21 years of age who will be recipients of mismatched-related or phenotypically similar unrelated donor marrow grafts for leukemia.  In this Phase I dose escalation study, spontaneous lymphoblastoid cell lines will be established that express the same range of Epstein-Barr Virus (EBV) encoded proteins as the recipient.  These EBV-specific cell lines will be transduced with LNL6 or G1Na and readministered at the time of bone marrow transplant.  This study will determine:  (1) survival and expansion of these EBV-specific cell lines in vivo, (2) the ability of these adoptively transferred cells to confer protection against EBV infection, and (3) appropriate dosage and administration schedules. (Protocol #9303-038)

 

Appendix D-45.  Drs. Robert W. Wilmott and Jeffrey Whitsett, Children's Hospital Medical Center, Cincinnati, Ohio, and Dr. Bruce Trapnell, Genetic Therapy, Inc., Gaithersburg, Maryland, may conduct experiments on 15 cystic fibrosis (CF) patients who have mild to moderate disease 21 years of age.  The replication-deficient type 5 adenovirus vector, Av1CF2, will be administered to the nasal and lobar bronchial respiratory tract of patients.  This study will demonstrate the:  (1) expression of normal cystic fibrosis transmembrane conductance regulator (CFTR) mRNA in vivo, (2) synthesis of CFTR protein, and (3) correction of epithelial cell cAMP dependent Cl- permeability.  The pharmacokinetics of CFTR expression and ability to re-infect the respiratory tract with AvCF2 will be determined.  Systemic and local immunologic consequences of Av1CF2 infection, the time of viral survival, and potential for recombination or complementation of the virus will be monitored. (Protocol #9303-041)

 

Appendix D-46.  Dr. James M. Wilson of the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, may conduct experiments on 20 adult patients with advanced cystic fibrosis lung disease.  An isolated segment of the patients' lung will be transduced with the E1 deleted, replication-incompetent adenovirus vector, Ad.CB-CFTR using a bronchoscope for gene delivery.  Ad.CB-CFTR contains the human gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein.  Pulmonary biopsies will be obtained by bronchoscopy at 4 days, 6 weeks, and 3 months following treatment.  Patients will be monitored for evidence of CFTR gene transfer and expression, immunological responses to CFTR or adenovirus proteins, and toxicity. (Protocol #9212-035)

 

Appendix D-47.  Dr. Hilliard F. Seigler of Duke University Medical Center, Durham, North Carolina, may conduct experiments on 20 patients with disseminated malignant melanoma.  Autologous tumor cells will be transduced with a retroviral vector, pHuγ-IFN, that contains the gene encoding human γ-IFN.  Following lethal irradiation, the transduced cells will be readministered to patients for the purpose of generating cytotoxic T cells that are tumor specific along with the up-regulation of Class I major histocompatibility antigens.  Patients will be monitored for clinical regression of tumors and generation of tumor-specific cytotoxic T lymphocytes. (Protocol #9306-043)

 

Appendix D-48.  Drs. Stefan Karlsson and Cynthia Dunbar of the National Institutes of Health, Bethesda, Maryland, and Dr. Donald B. Kohn of the Children’s Hospital of Los Angeles, Los Angeles, California, may conduct experiments on 10 patients with Gaucher disease.  CD34(+) hematopoietic stem cells will be isolated from bone marrow or from peripheral blood treated with granulocyte-colony stimulating factor.  CD34(+) cells will be transduced with a retrovirus vector, G1Gc, containing cDNA encoding human glucocerebrosidase and administered intravenously.  Patients will be monitored for toxicity and glucocerebrosidase expression. (Protocol #9306-047)

 

Appendix D-49.  Dr. Gary J. Nabel of the University of Michigan Medical Center, Ann Arbor, Michigan, may conduct experiments on 12 patients with AIDS to be divided into 4 experimental groups.  CD4(+) lymphocytes will be isolated from peripheral blood and transduced with Rev M10, a transdominant inhibitory mutant of the rev gene of the human immunodeficiency virus (HIV).  Transduction of the rev mutant will be mediated either by the retrovirus vector, PLJ-cREV M10, or by particle-mediated gene transfer of plasmid DNA.  Patients will be monitored for survival of the transduced CD4(+) cells by polymerase chain reaction and whether Rev M10 can confer protection against HIV infection to CD4(+) cells. (Protocol #9306-049)

 

Appendix D-50.  Dr. Gary J. Nabel of the University of Michigan Medical Center, Ann Arbor, Michigan, may conduct experiments on 24 patients with advanced cancer.  Patients will undergo in vivo transduction with DNA/liposome complexes containing genes encoding the HLA-B7 histocompatibility antigen and beta-2 microglobulin in a non-viral plasmid.  These DNA/liposome complexes will be administered either by intratumoral injection or catheter delivery.  Patients will be monitored for enhanced immune responses against tumor cells, and safe and effective doses will be determined. (Protocol #9306-045)

 

Appendix D-51.  Dr. John A. Barranger of the University of Pittsburgh, Pittsburgh, Pennsylvania, may conduct experiments on 5 patients with Gaucher disease.  The CD34(+) hematopoietic stem cells will be isolated from peripheral blood and transduced in vitro with the retrovirus vector, N2-Sv-GC, encoding the glucocerebrosidase (GC) enzyme.  Following reinfusion of the transduced cells, patients will be monitored by PCR analysis for GC expression in peripheral blood leukocytes.  Patients currently receiving GC replacement therapy and who demonstrate clinical responsiveness will be withdrawn from exogenous GC therapy.  Patients not previously treated with exogenous GC, will be monitored for clinical reversal of lipid storage symptoms. (Protocol #9306-046)

 

Appendix D-52.  Dr. Robert Walker of the National Institutes of Health, Bethesda, Maryland, may conduct experiments on 12 HIV-infected patients who have a seronegative identical twin.  CD4(+) and CD8(+) cells will be isolated from the seronegative twin and induced to polyclonal proliferation with anti-CD3 and interleukin-2.  The enriched population of cells will be transduced with either LNL6 or G1Na, which contain the neoR gene.  The transduced cells will be expanded in tissue culture and administered to the HIV-infected twin.  Patients will be monitored for immune function and the presence of marked cells. (Protocol #9209-026)

 

Appendix D-53.  Dr. Corey Raffel of the Children’s Hospital Los Angeles, Los Angeles, California, and Dr. Kenneth Culver of Iowa Methodist Medical Center, Des Moines, Iowa, may conduct experiments on 30 patients between 2 and 18 years of age with recurrent malignant astrocytoma.  Fifteen patients will be accrued into this study initially.  If at least one patient responds to therapy, an additional 14 patients will be treated.  Patients with either surgically accessible or non-accessible tumors will be treated with the vector producing cell line (PA317) carrying the retrovirus vector, G1TkSvNa.  This vector will transduce tumor cells in vivo with the Herpes simplex thymidine kinase (HS-tk) gene that renders the cells sensitive to killing by ganciclovir.  Surgically accessible patients will undergo surgical debulking of their tumor followed by repeated administration of the HS-tk vector producer cells into the tumor bed.  Children with unresectable tumors will undergo stereotaxic injection of vector producer cells into tumors. (Protocol #9306-050)

 

Appendix D-54.  Dr. Jeffrey E. Galpin of the University of Southern California, Los Angeles, California, and Dr. Dennis A. Casciato of the University of California, Los Angeles, California, may conduct experiments on 15 HIV(+) asymptomatic patients.  Patients will receive 3 monthly intramuscular injections of the retrovirus vector (N2IIIBenv) encoding the HIV-1 IIIB envelope protein.  Patients will be monitored for acute toxicity, CD4 levels, HIV-specific CTL responses, and viral burdens. (Protocol #9306-048)

 

Appendix D-55.  Drs. Charles Hesdorffer and Karen Antman of Columbia University College of Physicians and Surgeons, New York, New York, may conduct experiments on 20 patients with advanced breast, ovary, and brain cancer.  CD34(+) hematopoietic stem cells will be isolated from bone marrow, transduced with the retrovirus vector, PHaMDR1/A, and readministered to patients.  Patients will be monitored for the presence and expression of the MDR-1 gene.  The investigators will determine whether MDR-1 expression increases following chemotherapy. (Protocol #9306-051)

 

Appendix D-56.  Dr. Enzo Paoletti of Virogenetics Corporation, Troy, New York, may conduct experiments with poxvirus vectors NYVAC, ALVAC, and TROVAC at Biosafety Level 1.

 

Appendix D-57.  Drs. Richard C. Boucher and Michael R. Knowles of the University of North Carolina, Chapel Hill, North Carolina, may conduct experiments on 9 patients (18 years old or greater) with cystic fibrosis to test for the safety and efficacy of an E1-deleted recombinant adenovirus containing the cystic fibrosis transmembrane conductance regulator (CFTR) cDNA, Ad.CB-CFTR.  A single dose of 108, 3 x 109 or 1011 pfu/ml will be administered to the nasal cavity of 3 patients in each dose group.  Patients will be monitored by nasal lavage and biopsy to assess safety and restoration of normal epithelial function. (Protocol #9303-042)

 

Appendix D-58.  Dr. Joyce A. O'Shaughnessy of the National Institutes of Health, Bethesda, Maryland, may conduct experiments on 18 patients (18-60 years old) with Stage IV breast cancer who have achieved a partial or complete response to induction chemotherapy.  This study will determine the feasibility of obtaining engraftment of CD34(+) hematopoietic stem cells transduced by a retroviral vector, G1MD, and expressing a cDNA for the human multi-drug resistance-1 (MDR-1) gene following high dose chemotherapy, and whether the transduced MDR-1 gene confers drug resistance to hematopoietic cells and functions as an in vivo dominant selectable marker.  Patients will be monitored for evidence of myeloprotection and presence of the transduced MDR-1 gene." (Protocol #9309-054)

 

Appendix D-59.  Drs. Larry E. Kun, R. A. Sanford, Malcolm Brenner, and Richard L. Heideman of St. Jude Children's Research Hospital, Memphis, Tennessee, and Dr. Edward H. Oldfield of the National Institutes of Health, Bethesda, Maryland, may conduct experiments on 6 patients (3-21 years old) with progressive or recurrent malignant supratentorial tumors resistant to standard therapies.  Mouse cells producing the retroviral vector containing the herpes simplex thymidine kinase  gene (G1TKSVNa) will be instilled into the tumor areas via multiple stereotactically placed cannulas.  Patients will be treated with ganciclovir to eliminate cells expressing the transduced gene.  Patients will be monitored for central nervous system, hematologic, renal or other toxicities, and for anti-tumor responses by magnetic resonance imaging studies. (Protocol #9309-055)

 

Appendix D-60.  The physical containment level may be reduced from Biosafety Level 3 to Biosafety Level 2 for a Semliki Forest Virus (SFV) vector expression system of Life Technologies, Inc., Gaithersburg, Maryland.

 

Appendix D-61.  Dr. Albert B. Deisseroth of the University of Texas MD Anderson Cancer Center, Houston, Texas, may conduct experiments on 10 patients (16 to 60 years of age) with chronic lymphocytic leukemia.  Autologous peripheral blood and bone marrow cells will be removed from patients following chemotherapy and marked by transduction with two distinguishable retroviral vectors, G1Na and LNL6, containing the neomycin resistance gene.  The gene marked cells will be reinfused into patients to determine the efficiency of bone marrow purging and the origin of relapse following autologous bone marrow transplantation. (Protocol #9209-030)

 

Appendix D-62.  Dr. Jonathan Simons of the Johns Hopkins Oncology Center, Baltimore, Maryland, may conduct experiments on 26 patients (18 years of age) with metastatic renal cell carcinoma to evaluate the safety and tolerability of intradermally injected autologous irradiated tumor cells transduced with the retrovirus vector, MFG, containing the human granulocyte-macrophage colony stimulating factor gene.  Acute and long-term clinical toxicities and in vitro and in vivo induction of specific anti-tumor immune responses will be monitored. (Protocol #9303-040)

 

Appendix D-63.  Dr. Albert B. Deisseroth of the University of Texas MD Anderson Cancer Center, Houston, Texas, may conduct experiments on 20 patients (18 and 60 years old) with ovarian cancer.  A murine viral vector was constructed from the third generation of L series retroviruses with the insert of the human multi-drug resistance-1 (MDR-1) transduced gene.  The investigators will assess the safety and feasibility of administering CD34 (+) autologous peripheral blood and bone marrow cells.  Patients will be monitored for the presence of the MDR-1 gene and for the effect of gene transfer on hematopoietic function following the transplantation. (Protocol #9306-044)

 

Appendix D-64.  Dr. Joseph Ilan of the Case Western Reserve University School of Medicine and University Hospital of Cleveland, Cleveland, Ohio, may conduct experiments on 12 patients (18 years of age) with advanced brain cancer.  Human malignant glioma tumor cells will be cultured, transfected with Epstein-Barr virus (EBV)-based vector, anti-Insulin growth factor-I, lethally irradiated, and injected subcutaneously into patients in an attempt to express antisense Insulin growth factor-1.  Patients will be monitored for toxicity and immunologic responses to the vector. (Protocol #9306-052)

 

Appendix D-65.  Drs. James S. Economou and John Glaspy of the University of California, Los Angeles, California, may conduct experiments on 30 patients (18 to 70 years of age) with metastatic melanoma.  A human melanoma cell line (M-24) will be transduced with the retroviral vector, G1NaCvi2, expressing the human interleukin-2 (IL-2) gene.  The IL-2 producing cells will be mixed with the patient's autologous tumor cells, irradiated, and injected subcutaneously in an attempt to enhance the tumor-specific immunologic response.  Patients will be monitored for toxicity, in vitro and in vivo immunologic responses, and clinical anti-tumor effects. (Protocol #9309-058)

 

Appendix D-66.  Drs. Peter Cassileth, Eckhard R. Podack, and Kasi Sridhar of the University of Miami, and Niramol Savaraj of the Miami Veterans Administration Hospital, Miami, Florida, may conduct experiments on 12 patients ( 18 years of age) with limited stage small cell lung cancer.  Autologous tumor cells will be removed, expanded in culture, and transduced by lipofection with the BMG-Neo-hIL2 vector (derived from bovine papilloma virus).  The objective of this protocol is to demonstrate the safety and efficacy of administering IL-2 transduced autologous tumor cells in an attempt to stimulate a tumor-specific cytotoxic T lymphocyte (CTL) response, and to determine the quantity and characteristics of the CTL that have been generated. (Protocol #9309-053)

 

Appendix D-67.  Drs. Edward H. Oldfield and Zvi Ram of the National Institutes of Health, Bethesda, Maryland, may conduct experiments on 20 patients (18 years of age) with leptomeningeal carcinomatosis.  The patients will receive intraventricular or subarachnoid injection of murine vector producing cells containing the retroviral vector, G1Tk1SvNa.  Tumor cells expressing the herpes simplex thymidine kinase gene will be rendered sensitive to killing by subsequent administration of ganciclovir.  Patients will be monitored for safety and anti-tumor response by magnetic resonance imaging (MRI) and cerebral spinal fluid cytological analysis. (Protocol #9312-059)

 

Appendix D-68.  Drs. Tapas K. Das Gupta and Edward P. Cohen of the University of Illinois College of Medicine, Chicago, Illinois, may conduct experiments on 12 subjects who differ in at least 3 out of 6 alleles at the Class I histocompatibility locus (18 years of age) with Stage IV malignant melanoma.  The subjects will be immunized with a lethally irradiated allogeneic human melanoma cell line transduced with the human interleukin-2 expressing retroviral vector, pZipNeoSv-IL-2.  Subjects will be monitored for toxicity, induction of B and T cell antitumor responses in vitro and in vivo, and any clinical evidence of antitumor effect. (Protocol #9309-056)

 

Appendix D-69A.  Dr. Michael J. Welsh of the Howard Hughes Medical Institute, Iowa City, Iowa, may conduct experiments on 20 patients (18 years of age) with cystic fibrosis.  The investigator will administer increasing doses of either of the two adenovirus vectors, AD2/CFTR-1 or AD2-ORF6/PGK-CFTR, to the nasal epithelium of 10 patients (1 nostril) or maxillary sinus epithelium of 10 patients (1 maxillary sinus).  Patients will be isolated for a period of 24 hours following vector administration; however, if 1 patient demonstrates secreted virus at 24 hours, the investigator will notify the Recombinant DNA Advisory Committee for reconsideration of the isolated period.  Patients will be assessed for the safety and efficacy of multiply administration of adenovirus vectors encoding the cystic fibrosis transmembrane conductance regulator (CFTR) gene.  (Protocol #9312-067)

 

Appendix D-69B.  Dr. Richard Haubrich of the University of California at San Diego Treatment Center, San Diego, California, may conduct experiments on 25 human immunodeficiency virus (HIV)-infected, seropositive, asymptomatic subjects (18 to 65 years of age).  Subjects will receive 3 monthly intramuscular injections of the retroviral vector, N2/IIIB env/rev, which encodes for HIV-1 IIIB env/rev proteins.  The objective of the study is to induce HIV-1- specific CD8(+) cytotoxic T lymphocyte and antibody responses in order to eliminate HIV-infected cells and residual virus.  This Phase I/II study will evaluate acute toxicity, identify long-term treatment effects, and evaluate the disease progression. (Protocol #9312-062)

 

Appendix D-70.  Dr. Mario Sznol of the National Institutes of Health, Frederick, Maryland, may conduct experiments on 50 subjects (18 years of age) with advanced stage melanoma.  Subjects will receive subcutaneous injections of lethally irradiated allogeneic melanoma cells that have been transduced by lipofection with the plasmid DNA vector, CMV-B7, derived from bovine papilloma virus to express the human B7 antigen.  The B7 antigen, which binds to the CD28 receptor of T cells, will serve as a co-stimulatory signal to elicit an antitumor immune response.  Subjects will be monitored for induction of cytotoxic T lymphocyte, antitumor responses in vitro and in vivo and any clinical evidence of antitumor effect. (Protocol #9312-063)

 

Appendix D-71.  Dr. Joseph Rubin of the Mayo Clinic, Rochester, Minnesota, may conduct experiments on 15 subjects with hepatic metastases from advanced colorectal cancer ( 18 years of age).  Subjects will receive intratumoral hepatic injections of the plasmid DNA/lipid complex, pHLA-B7/β-2 microglobulin, expressing a heterodimeric cell surface protein consisting of the HLA-B7 histocompatibility antigen and β-2 microglobulin.  Subjects must be HLA-B7 negative.  The objective of this study is to determine a safe and effective dose of the DNA/lipid complex.  Subjects will be monitored for antigen-specific immune responses and in vivo HLA-B7 expression. (Protocol #9312-064)

 

Appendix D-72.  Dr. Nicholas J. Vogelzang of the University of Chicago Medical Center, Chicago, Illinois, may conduct experiments on 15 subjects with metastatic renal cell carcinoma 18 years of age.  Subjects will receive intratumoral injections of the plasmid DNA/liposome vector pHLA-B7/β-2 microglobulin, expressing a heterodimeric cell surface protein consisting of the HLA-B7 histocompatibility antigen and β-2 microglobulin.  Subjects must be HLA-B7 negative.  Subjects will be monitored for antigen-specific immune responses and in vivo HLA-B7 expression. (Protocol #9403-071)

 

Appendix D-73.  Dr. Evan M. Hersh of the Arizona Cancer Center and Drs. Emmanuel Akporiaye, David Harris, Alison T. Stopeck, Evan C. Unger, and James A. Warneke of the University of Arizona, Tucson, Arizona, may conduct experiments on 15 subjects with metastatic malignant melanoma 18 years of age.  Subjects will receive intratumoral injections of the plasmid DNA/liposome vector, pHLA-B7/β-2 microglobulin, expressing a heterodimeric cell surface protein consisting of the HLA-B7 histocompatibility antigen and β-2 microglobulin.  Subjects must be HLA-B7 negative.  Subjects will be monitored for antigen-specific immune responses and in vivo HLA-B7 expression. (Protocol #9403-072)

 

Appendix D-74.  Dr. Ralph Freedman of MD Anderson Cancer Center, Houston, Texas, may conduct gene marking experiments on 9 subjects with ovarian carcinoma or peritoneal carcinomatosis ( 16 years of age).  Autologous CD3(+)/CD8(+) tumor infiltrating lymphocyte derived T cells will be transduced with the retroviral vector G1Na that encodes for neoR.  Subjects will receive intraperitoneal administration of bulk expanded transduced and nontransduced T cells and recombinant interleukin-2.  Previously documented tumor sites and normal tissues will be monitored for neoR and the proportion of CD3(+)/CD8(+) T cells will be determined.  (Protocol #9406-075)

 

Appendix D-75.  Drs. Helen Heslop, Malcolm Brenner, and Robert Krance of St. Jude Children’s Research Hospital, Memphis, Tennessee, may conduct gene marking experiments on 20 subjects undergoing autologous bone marrow transplantation for therapy of leukemia or solid tumor (< 21 years of age).  The distinguishable retroviral vectors, LNL6 and G1Na (both encoding neoR), will be used to determine the rate of reconstitution of untreated versus cytokine expanded CD34(+) selected autologous bone marrow cells.  (Protocol #9406-076)

 

Appendix D-76.  Drs. Albert Deisseroth, Gabriel Hortobagyi, Richard Champlin, and Frankie Holmes of MD Anderson Cancer Center, Houston, Texas, may conduct experiments on 10 fully evaluable subjects (maximum of 20 entered) with Stage III or IV breast cancer ( 18 and 60 years of age).  Subjects will receive autologous CD34(+) peripheral blood cells that have been transduced with the retroviral vector, pVMDR-1, which encodes the multi-drug resistance gene.  The objective of this study is to evaluate the safety and feasibility of transducing early hematopoietic progenitor cells with pVMDR-1 and to determine in vivo selection of chemotherapy resistant hematopoietic cells.  (Protocol #9406-077)

 

Appendix D-77.  Drs. Johnson M. Liu and Neal S. Young of the National Institutes of Health, Bethesda, Maryland, may conduct experiments on 6 patients with Fanconi anemia ( 5 years of age).  Subjects will receive autologous CD34(+) cells that have been transduced with the retroviral vector, FACC, which encodes the normal Fanconi anemia complementation group C gene.  The objective of this study is to determine whether autologous FACC transduced hematopoietic progenitor cells can be safely administered to subjects, the extent of engraftment, and correction of cell phenotype.  (Protocol #9406-078)

 

Appendix D-78.  Drs. Robert E. Sobol and Ivor Royston of the San Diego Regional Cancer Center, San Diego, California, may conduct experiments on 15 subjects with recurrent residual glioblastoma multi-forme ( 18 years of age). Subjects will receive subcutaneous injections of autologous tumor cells that have been lethally irradiated and transduced with the retroviral vector, G1NaCvi2.23, which encodes for interleukin-2.  Subjects will be monitored in vitro for cellular and humoral antitumor responses and in vivo for antitumor activity.  (Protocol #9406-080)

 

Appendix D-79.  Dr. Alfred E. Chang of the University of Michigan Medical Center, Ann Arbor Michigan, may conduct gene marking experiments on 15 subjects with metastatic melanoma ( 18 years of age).  Subjects will undergo adoptive immunotherapy of anti-CD3/interleukin-2 activated lymph node cells that have been primed in vivo with tumor cells that have been transduced with the retrovirus vector, GBAH4, encoding the gene for interleukin-4.  The investigator will evaluate the antitumor efficacy and in vivo immunological reactivity in subjects receiving adoptively transferred T cells, and the in vitro immunological reactivities of the activated T cells that might correlate with their in vivo antitumor function.  (Protocol #9312-065)

 

Appendix D-80.  Dr. Robert Walker of the National Institutes of Health, Bethesda, Maryland, may conduct gene marking experiments on 40 HIV(+) subjects ( 18 years of age).  The investigator may also enter an additional number of subjects (to be determined by the investigator) who will receive a single administration of 1 x 107 HIV-specific CD8(+) cells.  The investigator will:  (1) Assess the safety and tolerance of the adoptive transfer of anti-HIV cytotoxic, syngeneic, CD8(+) peripheral blood lymphocytes that have been transduced with the retrovirus

vector, rkat4svgF3e-, that encodes for a universal chimeric T cell receptor.  (2) Determine the longevity of the genetically marked CD8(+) lymphocytes in the subject's peripheral blood.  (Protocol #9403-069)

 

Appendix D-81.  Dr. Joseph Rosenblatt of the University of California, Los Angeles, California, and Dr. Robert Seeger of Children’s Hospital, Los Angeles, California, may conduct gene transfer experiments on 18 subjects with neuroblastoma ( 21 years of age).  Patients at high risk of relapse with minimal or no detectable disease following myeloablative therapy and autologous bone marrow transplantation, or patients with progressive or persistent disease despite conventional therapy will be serially immunized with autologous or allogeneic neuroblastoma cells transduced to express γ interferon.  Cells will be transduced with the retroviral vector, pHuγ-IFN, encoding the human gene for γ interferon and lethally irradiated prior to use as an immunogen.  The objectives of the study are: (1) to determine the maximum tolerable dose of transduced cells; (2) to determine the local, regional, and systemic toxicities of injected cells; and (3) to determine the antitumor response in vivo as measured by standard clinical tests and immunocytologic evaluation of marrow metastases.  (Protocol #9403-068)

 

Appendix D-82.  Dr. Kenneth L. Brigham of Vanderbilt University, Nashville, Tennessee, may conduct gene transfer experiments on 10 subjects ( 21 years of age) in two different patient protocols (5 for each protocol).  Both protocols will use the same DNA/liposome preparations to deliver a plasmid DNA construct, pCMV4-AAT, encoding human alpha-1 antitrypsin gene driven by a cytomegalovirus promoter.  In patients scheduled for elective pulmonary resection, the DNA/liposome complexes will be instilled through a fiber optic bronchoscope into a subsegment of the lung.  Tissues of the lung will be obtained at the time of surgery.  Transgene expression will be assessed by immunohistochemistry, in situ hybridization, and Western and Northern blot analyses.  The effect of DNA/liposome complex administration on the histological appearance of the lung will also be evaluated.  In patients with alpha-1 antitrypsin deficiency, the DNA/liposome complexes will be instilled into the nostril.  Transgene expression will be determined in cells obtained by nasal lavage and nasal scraping, and the time course of transgene expression will be measured.  The secretion of the alpha-1 antitrypsin protein in nasal fluid will be determined.  Histological appearance of nasal mucosa will also be examined.  The study will assess safety and feasibility of gene delivery to the human respiratory tract.  (Protocol #9403-070)

 

Appendix D-83.  Dr. H. Kim Lyerly of Duke University Medical Center, Durham, North Carolina, may conduct gene transfer experiments on 20 subjects with refractory or recurrent metastatic breast cancer ( 18 years of age).  Autologous breast cancer cells will be transduced with the DNA/liposome complex, pMP6-IL2, containing a plasmid DNA vector derived from adeno-associated virus (AAV) that expresses the gene for human interleukin-2.  Subjects will receive 4 subcutaneous injections of lethally irradiated tumor cells transduced with the DNA/liposome complex prior to injection.  The objective of this study is to:  (1) evaluate the safety and toxicity of the treatment, (2) determine the immunological effects, (3) determine the duration of clinical responses, and (4) measure patient survival.  (Protocol #9409-086)

 

Appendix D-84.  Drs. Flossie Wong-Staal, Eric Poeschla, and David Looney of the University of California at San Diego, La Jolla, California, may conduct gene transfer experiments on 6 subjects (18 and 65 years of age) infected with human immunodeficiency virus-1 (HIV-1).  Autologous CD4(+) T lymphocytes will be transduced ex vivo with the retroviral vector, pMJT, expressing a hairpin ribozyme that cleaves the HIV-1 RNA in the 5' leader sequence.  The transduced cells will be expanded and reinfused into the patients.  The objectives of the study are: (1) to evaluate safety of reinfusing the transduced lymphocytes, (2) to compare (in vivo) the kinetics and survival of ribozyme-transduced cells with that of cells transduced with a control vector, (3) to determine in vivo expression of the ribozyme sequences in transduced lymphocytes, (4) to determine whether host immune responses directed against the transduced cells will occur in vivo, and (5) to obtain preliminary data on the effects of ribozyme gene therapy on in vivo HIV mRNA expression, viral burden and CD4(+) lymphocyte levels.  (Protocol #9309-057)

 

Appendix D-85.  Dr. Friedrich Schuening of the Fred Hutchinson Cancer Research Center, Seattle, Washington, may conduct gene transfer experiments on 10 subjects (18 years of age) with Type I Gaucher's disease.  The peripheral blood repopulating cells (mobilized by patient pretreatment with recombinant granulocyte colony-stimulating factor) will be harvested and CD34(+) cells selected.  CD34(+) cells will be transduced ex vivo with the retroviral vector, LgGC, that encodes human glucocerebrosidase cDNA.  Following transduction, the transduced cells will be infused into the patient without myeloablative treatment.  The primary endpoint of this study is to examine the safety of infusing CD34(+) cells transduced with the human glucocerebrosidase cDNA.  Patients will be monitored for persistence and expression of the glucocerebrosidase gene in hematopoietic cells.  (Protocol #9312-061)

 

Appendix D-86.  Dr. Terence R. Flotte of the Johns Hopkins Children's Center, Baltimore, Maryland, may conduct gene transfer experiments on 16 subjects (18 years of age) with mild cystic fibrosis (CF).  An adeno-associated virus (AAV) derived vector, encoding cystic fibrosis transmembrane conductance regulator (CFTR) gene, (tgAAVCF), will be administered to nasal (direct) and airway (bronchoscope) epithelial cells.  This is a dose escalation study involving 8 cohorts.  Each subject will receive both intranasal and bronchial administration of the adenoviral vector at 4 escalating doses.  Nasal doses will range between 1 x 106 and 1 x 109 pfu.  Lung administration will range between 1 x 107 and 1 x 1010 pfu.  The primary goal of the study is to assess the safety of vector administration.  Respiratory and nasal epithelial cells will be evaluated for gene transfer, expression, and physiologic correction.  (Protocol #9409-083)

 

Appendix D-87.  Drs. Jeffrey M. Isner and Kenneth Walsh of St. Elizabeth's Medical Center, Tufts University, Boston, Massachusetts, may conduct gene transfer experiments on 12 subjects (40 years of age) with peripheral artery disease (PAD).  A plasmid DNA vector, phVEGF165, encoding the human gene for vascular endothelial growth factor (VEGF) will be used to express VEGF to induce collateral neovascularization.  Percutaneous arterial gene transfer will be achieved using an angioplasty catheter with a hydrogel coated balloon to deliver the plasmid DNA vector to the artery.  The objectives of the study are:  (1) to determine the efficacy of arterial gene therapy to relieve rest pain and/or heal ischemic ulcers of the lower extremities in patients with PAD; and (2) to document the safety of the phVEGF arterial gene therapy for therapeutic angiogenesis.  Subjects will undergo anatomic and physiologic examination to determine the extent of collateral artery development following phVEGF arterial gene therapy.  (Protocol #9409-088)

 

Appendix D-88A.  Dr. Ronald G. Crystal of New York Hospital-Cornell Medical Center, New York, New York, may conduct gene transfer experiments on 26 patients ( 15 years of age) with cystic fibrosis (CF).  A replication deficient recombinant adenovirus vector will be used to transduce epithelial cells of the large bronchi with the E1/E3 deleted type 5 adenovirus vector, AdGVCFTR.10, which encodes the human cystic fibrosis transmembrane conductance regulator (CFTR) gene.  The objective of this study is to define the safety and pharmacodynamics of CFTR gene expression in airway epithelial cells following single administration of escalating doses to the vector.  If single administration is determined to be safe, subjects will undergo repeat administration to localized areas of the bronchi.  (Protocol #9409-085)

 

Appendix D-88B.  Drs. Eric J. Sorscher and James L. Logan of the University of Alabama, Birmingham, Alabama, may conduct gene transfer experiments on 9 subjects (18 years of age) with cystic fibrosis (CF).  The normal human cystic fibrosis transmembrane conductance regulator (CFTR) gene will be expressed by a plasmid DNA vector, pKCTR, driven by the simian virus-40 (SV40) early gene promoter.  The CFTR DNA construct will be delivered by cationic liposome-based gene transfer to nasal epithelial cells.  The objectives of the study are to:  (1) evaluate the safety of lipid-mediated gene transfer to nasal epithelial cells (including local inflammation and mucosal tissue); and (2) evaluate efficacy as determined by correction of the chloride ion transport defect, and wild-type CFTR mRNA and protein expression. (Protocol #9312-066)

 

Appendix D-89.  Dr. Steven M. Albelda of the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, may conduct gene transfer experiments on 12 subjects with advanced mesothelioma.  The adenovirus vector encoding the Herpes simplex virus thymidine kinase (HSV-TK) gene, H5.020RSVTK, will be administered through a chest tube to the pleural cavity.  Tumor biopsies will be assayed for gene transfer and expression.  Subjects will be monitored for immunological responses to the adenovirus vector.  Ganciclovir will be administered intravenously 14 days following vector administration.  The primary objective of this Phase I study is to evaluate the safety of direct adenovirus vector gene delivery to the pleural cavity of patients with malignant melanoma. (Protocol #9409-090)

 

Appendix D-90.  Drs. Jeffrey Holt and Carlos B. Arteaga of the Vanderbilt University, Nashville, Tennessee, may conduct gene transfer experiments on 10 female patients (over 18 years of age) with metastatic breast cancer. Patient effusions from pleura or peritoneum will be drained and the fluid will be replaced with supernatant containing the retroviral vectors, XM6:antimyc or XM6:antifos, which express c-myc and c-fos antisense sequences, respectively, under the control of a mouse mammary tumor virus promoter.  The objectives of this study are to:  (1)  assess uptake and expression of the vector sequences in breast cancer cells present in pleural and peritoneal fluids, and determine if this expression is tumor specific, (2) assess the safety of localized administration of antisense retroviruses, and (3) monitor subjects for clinical evidence of antitumor response. (Protocol #9409-084)

 

Appendix D-91.  Dr. Jack A. Roth of MD Anderson Cancer Center, Houston, Texas, may conduct gene transfer experiments on 14 non-small cell lung cancer subjects (18 and 80 years of age) who have failed conventional therapy and who have bronchial obstruction.  LNSX-based retroviral vectors containing the β-actin promoter will be used to express: (1) the antisense RNA of the K-ras oncogene (LN-K-rasB), and (2) the wildtype p53 tumor suppressor gene (LNp53B).  Tumor biopsies will be obtained to characterized K-ras and p53 mutations.  Relative to their specific mutation, subjects will undergo partial endoscopic resection of the tumor bed followed by bronchoscopic administration of the appropriate retrovirus construct.  The objective of this study is to evaluate the safety and efficacy of intralesional administration of LN-K-rasB and LNp53 retrovirus constructs. (Protocol #9403-031)

 

Appendix D-92.  Drs. Robert E. Sobol and Ivor Royston of the San Diego Regional Cancer Center, San Diego, California, may conduct gene transfer experiments on 12 subjects (18 years of age) with metastatic colon carcinoma.  The autologous skin fibroblasts will be transduced with the retroviral vector, LNCX/IL-2, which encodes the gene for human interleukin-2 (IL-2).  In this dose-escalation study, subjects will receive subcutaneous injections of lethally irradiated autologous tumor cells.  The objectives of the study are to: (1) evaluate the safety of subcutaneous administration of LNCX/IL-2 transduced fibroblasts, (2) determine in vivo antitumor activity, and (3) monitor cellular and humoral antitumor responses.  (Protocol #9312-060)

 

Appendix D-93.  Dr. Michael Lotze of the University of Pittsburgh, Pittsburgh, Pennsylvania, may conduct gene transfer experiments on 18 subjects (18 years of age) with advanced melanoma, 6 with T-cell lymphoma, breast cancer, or head and neck cancer.  Subjects should have accessible cutaneous tumors, and have failed standard therapy.  Over 4 weeks, subjects will receive a total of 4 intratumoral injections of autologous fibroblasts transduced with the retrovirus vector, TFG-hIL-12-Neo.  This vector, which consists of the murine MFG backbone, expresses both the p35 and p40 subunits of interleukin-12 (IL-12) and the neoR selection marker.  The objectives of the study are to:  (1) define the local and systemic toxicity associated with peritumoral injections of gene-modified fibroblasts, (2) examine the local and systemic immunomodulatory effects of these injections, and (3) evaluate clinical antitumor efficacy.  (Protocol #9406-081)

 

Appendix D-94.  Drs. Evan Hersh, Emmanuel Akporiaye, David Harris, Alison Stopeck, Evan Unger, James Warneke, of the Arizona Cancer Center, Tucson, Arizona, may conduct gene transfer experiments on 25 subjects (18 years of age) with solid malignant tumors or lymphomas.  A plasmid DNA/lipid complex designated as VCL-1102 (IL-2 Plasmid DNA/DMRIE/DOPE) will be used to transduce the human gene for interleukin-2 (IL-2).  Patients with advanced cancer who have failed conventional therapy will undergo a procedure in which VCL-1102 is injected directly into the tumor mass to induce tumor-specific immunity.  The objectives of the study are to:  (1) determine safety and toxicity associated with escalating doses of VCL-1102; (2) confirm IL-2 expression in target cells; (3) determine biological activity and pharmacokinetics; and (4) determine whether IL-2 expression stimulates tumor regression in subjects with metastatic malignancies.  (Protocol #9412-095)

 

Appendix D-95.  Drs. Richard Morgan and Robert Walker of the National Institutes of Health, Bethesda, Maryland, may conduct gene transfer experiments on 48 human immunodeficiency virus (HIV) seropositive subjects (18 years of age).  This Phase I/II study involves identical twins (one HIV seropositive and the other HIV seronegative).  CD4(+) T cells will be enriched following apheresis of the HIV seronegative twin, induced to polyclonal proliferation with anti-CD3 and recombinant IL-2, transduced with either the LNL6/NeoR or G1Na/NeoR, and transduced with up to 2 additional retroviral vectors (G1RevTdSN and/or GCRTdSN(TAR)) containing potentially therapeutic genes (antisense TAR and/or transdominant Rev).  These T cell populations will be expanded 10 to 1,000 fold in culture for 1 to 2 weeks and reinfused into the HIV seropositive twin.  Subjects will receive up to 4 cycles of treatment using identical or different combinations of control and anti-HIV retrovirus vectors.  The relative survival of these transduced T cell populations will be monitored by vector-specific polymerase chain reaction, while the subjects' functional immune status is monitored by standard in vitro and in vivo assays.  (Protocol #9503-103)

 

Appendix D-96.  Dr. Harry L. Malech of the National Institutes of Health, Bethesda, Maryland, may conduct gene transfer experiments on 2 subjects 18 years of age (with or without concurrent serious infection), and 3 subjects 18 years of age (with or without concurrent serious infection) or minors 13-17 years of age who have concurrent serious infection who have chronic granulomatous disease (CGD).  CGD is an inherited immune deficiency disorder in which blood neutrophils and monocytes fail to produce antimicrobial oxidants (p47phox mutation) resulting in recurrent life-threatening infections.  Subjects will undergo CD34(+) mobilization with granulocyte colony stimulating factor (G-CSF).  These CD34(+) cells will be transduced with the retrovirus vector, MFG-S-p47phox, which encodes the gene for normal p47phox.  The objectives of this study are to:  (1) determine the safety of administering MFG-S-p47phox transduced CD34(+) cells, and (2) demonstrate increased functional oxidase activity in circulating neutrophils.  (Protocol #9503-104)

 

Appendix D-97.  Drs. Chris Evans and Paul Robbins of the University of Pittsburgh, Pittsburgh, Pennsylvania, may conduct gene transfer experiments on 6 subjects (18 and 76 years of age) with rheumatoid arthritis.  Rheumatoid arthritis is a chronic, progressive disease thought to be of autoimmune origin.  A gene encoding an interleukin-1 receptor antagonist protein (IRAP) will be delivered to the rheumatoid metacarpal-phalangeal joints to determine the autoimmune reactions can be interrupted.  The vector construct, DFG-IRAP, is based on the MFG murine retrovirus vector backbone, and encodes the human IRAP gene.  Synovial fibroblasts will be generated from the rheumatoid arthritic joint tissue obtained from patients who are scheduled to undergo surgery.  The fibroblasts will be transduced with the DFG-IRAP vector, and the transduced cells injected into the synovial space.  The synovial fluid and joint material will be collected 7 days later to determine the presence and location of the transduced synovial fibroblasts and the level of IRAP in the joint fluid.  (Protocol 9406-074)

 

Appendix D-98.  Dr. R. Scott McIvor of the University of Minnesota, Minneapolis, Minnesota, may conduct gene transfer experiments on 2 children with purine nucleoside phosphorylase (PNP) deficiency.  PNP deficiency results in severe T-cell immunodeficiency, an autosomal recessive inherited disease which is usually fatal in the first decade of life.  Autologous peripheral blood lymphocytes will be cultured in an artificial capillary cartridge in the presence of anti-CD3 monoclonal antibody and interleukin-2 and transduced with the retroviral vector, LPNSN-2, encoding human PNP.   Subjects will undergo bimonthly intravenous administration of transduced T cells for a maximum of 1 year.  The objectives of the study are to determine:  (1) the safety of intravenous administration of transduced T cells in children with PNP deficiency, (2) the efficiency of PNP gene transfer and duration of gene expression in vivo, and (3) the effect of PNP gene transfer on immune function.  (Protocol #9506-110)

 

Appendix D-99.  Drs. Nikhil C. Munshi and Bart Barlogie of the University of Arkansas School for Medical Sciences, Little Rock, Arkansas, may conduct gene transfer experiments on 21 subjects (>18 and <65 years of age) with relapsed or persistent multiple myeloma who are undergoing T cell depleted allogeneic bone marrow transplantation.  Donor peripheral blood lymphocytes will be cultured in vitro with interleukin-2 and anti-CD3 monoclonal antibody.  T cell depleted lymphocytes will be transduced with the retroviral construct, G1Tk1SvNa.7, which encodes the Herpes simplex virus thymidine kinase (HSV-TK) gene.  The transduced cells will be reinfused.  In this dose escalation study, 3 subjects will undergo cell-mediated gene transfer per cohort (maximum of 5 cohorts) until Grade III or IV Graft versus Host Disease (GVHD) is observed.  A maximum of 6 additional patients may be entered at that maximum tolerated dose.  The objectives of this study are to determine the:  (1) safety of transduced donor cell infusions, (2) effectiveness of donor cell infusions in decreasing the effects of severe GVHD, (3) effectiveness of donor cell infusions in prolonging multiple myeloma remission, and (4) effectiveness of ganciclovir in eliminating donor cells for the purpose of preventing the depletion of erythrocytes.  (Protocol #9506-107)

 

Appendix D-100.  Dr. Wayne A. Marasco of Dana-Farber Cancer Institute, Boston, Massachusetts, may conduct gene transfer experiments on 6 subjects (18 and 65 years of age) with human immunodeficiency virus type-1 (HIV-1).  Autologous lymphocytes from asymptomatic subjects will be transduced ex vivo with a retroviral vector, LNCs105, encoding the sFv105 antibody specific for the HIV-1 envelope protein.  An identical aliquot will be simultaneously transduced with a control retroviral vector lacking the sFv105 cassette.  Transduced cells will be reinfused into patients and the differential survival of both populations of CD4+ lymphocytes compared.  The objective of the study is to determine whether the intracellular expression of a human single chain antibody against HIV-1 envelope glycoprotein gp160 that blocks gp160 processing and the production of infectious virions can safely prolong the survival of CD4(+) lymphocytes in HIV-1-infected subjects.  (Protocol #9506-111)

 

Appendix D-101.  Dr. Henry Dorkin of the New England Medical Center, Boston, Massachusetts, and Dr. Allen Lapey of Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, propose to conduct gene transfer experiments on 16 subjects (18 years of age).  An E1/partial E4-deleted, replication-deficient, type 2 adenovirus vector, AD2/CFTR-2, will be used to deliver the human cystic fibrosis transmembrane conductance regulator (CFTR) gene by aerosol administration (nebulization) to the lung of CF patients.   Aerosol administration will be initiated only after initial safety data has been obtained from the lobar administration protocol (#9409-091).  This is a single administration dose-escalation study in which subjects will receive between 8 x 106 and 2.5 x 1010 pfu.  Subjects will be assessed for evidence of adverse, systemic, immune, inflammatory, or respiratory effects in response to AD2/CFTR-2.  Subjects will be monitored for virus shedding and transgene expression.  Health care workers present in the facility will be required to sign an Informed Consent document regarding the possibility of virus transmission.  (Protocol #9412-074)

 

Appendix D-102.  Drs. Charles J. Link and Donald Moorman of the Human Gene Therapy Research Institute, Des Moines, Iowa, may conduct gene transfer experiments on 24 female subjects (18 years of age) with refractory or recurrent ovarian cancer.  Subjects will undergo intraperitoneal delivery (via Tenkhoff catheter) of the vector producing cells (VPC), PA317/LTKOSN.2.  These VPC express the Herpes simplex virus thymidine kinase (HSV-TK) gene which confers sensitivity to killing by the antiviral drug, ganciclovir (GCV).  The LTKOSN.2 retrovirus vector is based on the LXSN backbone.  Two weeks following intraperitoneal delivery of the VPC, subjects will receive 5 mg/kg intravenous GCV twice daily for 14 days.  Subjects will receive between 1 x 105 and 1 x 108 VPC/kg in this dose escalation study.  Subjects will be evaluated by X-ray and peritoneoscopy of the abdomen for evidence of clinical response.  The objectives of this study are to determine the safety of intraperitoneal VPC administration.  (Protocol #9503-100)

 

Appendix D-103.  Dr. David T. Curiel of the University of Alabama, Birmingham, Alabama, may conduct gene transfer experiment of 15 subjects (18 years of age) with metastatic colorectal cancer.  Subjects will receive intramuscular injection of the polynucleotide vaccine, pGT63, which is a plasmid DNA vector expressing carcinoembryonic antigen (CEA) and hepatitis B surface antigen (HBsAg).  The objectives of the study are to:   (1) characterize the immune response to CEA and HBsAg following a single intramuscular injection and following 3 consecutive intramuscular injections, and (2) determine the safety of intramuscular injection of the plasmid DNA vector at doses ranging between 0.1 to 1.0 milligrams (single dose) and 0.9 to 3.0 milligrams (total multidose). (Protocol #9506-073)

 

Appendix D-104.  Dr. Chester B. Whitley of the University of Minnesota, Minneapolis, Minnesota, may conduct gene transfer experiments on two adult subjects (18 years of age or older) with mild Hunter syndrome (Mucopolysaccharidosis Type II).  The autologous peripheral blood lymphocytes will be transduced ex vivo with the retroviral vector, L2SN, encoding the human cDNA for iduronate-2-sulfatase (IDS).  The transduced lymphocytes will be reinfused into the patients on a monthly basis.  The study will determine the frequency of peripheral blood lymphocyte transduction and the half-life of the infused cells.  Evaluation of patients will include measurement of blood levels of IDS enzyme, assessment of metabolic correction by urinary glycosaminoglycan levels, clinical response of the disease, and monitoring for potential toxicity.  This Phase I study is to demonstrate the safety of the L2SN-mediated gene therapy and to provide a preliminary evaluation of clinical efficacy.  (Protocol #9409-087)

 

Appendix D-105.  Drs. James Economou, John Glaspy, and William McBride of the University of California, Los Angeles, California, may conduct gene transfer experiments on 25 subjects (18 years of age) with metastatic melanoma.  The protocol is an open label, Phase I trial to evaluate the safety and immunological effects of administering lethally irradiated allogeneic and autologous melanoma cells transduced with the retroviral vector, IL-7/HyTK, which encodes the gene for human interleukin-7 (IL-7).  Subjects will receive 1 x 107 irradiated unmodified autologous tumor cells in combination with escalating doses of IL-7/HyTK transduced allogeneic melanoma cells (M24 cell line).  The number of M24 cells administered will be adjusted based on the level of IL-7 expression.  Subjects will receive 3 biweekly subcutaneous injections of M24 cells expressing 10, 100, or 1000 nanograms of IL-7/hour in vivo.  A final cohort of 5 subjects will receive IL-7/HyTK transduced autologous cells.  Subjects will be monitored for antitumor activity by skin tests, biopsy analysis, tumor-specific antibody activity, and cytotoxic T lymphocyte precursor evaluation.  Non-immunologic parameters will also be monitored. (Protocol #9503-101)

 

Appendix D-106.  Dr. Jack A. Roth, MD Anderson Cancer Center, may conduct gene transfer experiments on 42 subjects (18 years of age) with refractory non-small cell lung cancer (NSCLC).  Subjects will receive direct intratumoral injection of a replication-defective type 5 adenovirus vector, AD5CMV-p53, to deliver the normal human p53 tumor suppressor gene.  The E1 region of AD5CMV-p53 has been replaced with a p53 expression cassette containing the human cytomegalovirus promoter (CMV).  Subjects will be divided into 2 treatment groups:  (1) 21 subjects will receive Ad5CMV-p53 alone, and (2) 21 subjects will receive Ad5CMV-p53 in combination with cisplatin.  Following vector administration, subjects will be isolated for 96 hours during which time, assays will be conducted to demonstrate the lack of shedding of adenovirus vector.  The objectives of this study are determine:  (1) the maximum tolerated dose of AD5CMV-p53, (2) qualitative and quantitative toxicity related to vector administration, and (3) biologic activity.

 

Prior to administration, adenovirus vector stocks will be screened for p53 mutants using the SAOS osteosarcoma cell assay that was submitted by Dr. Roth on June 23, 1995.  This biologic assay compares the activity of a standard stock of Adp53 vector to the activity of newly produced stocks.  The standard stock of Adp53 will be defined as mediating cell death in 100% of SAOS cells (human osteosarcoma cell line with homozygous p53 deletion) at an MOI of 50:1 (titer > 5 x 1010) on day 5 of culture.  The sensitivity of the assay for detecting inactive (presumed mutant) Adp53 vector will be determined by adding increasing amounts of Adluc (control adenovirus vector containing the luciferase gene) to the Adp53 stock to determine the percentage of inactive vector required to decrease growth inhibition of SAOS cells mediated by Adp53.  The test lot of Adp53 will be tested for its ability to inhibit SAOS in a 5 day assay.  Significant loss of inhibitory activity compared with the standard would indicate an unacceptable level of inactive (presumed mutant) vector. (Protocol #9406-079)

 

Appendix D-107A.  Dr. Gary Clayman. M.D. Anderson Cancer Center, Houston, Texas, may conduct gene transfer experiments on 21 subjects (18 years of age) with refractory squamous cell carcinoma of the head and neck.  Subjects will receive direct intratumoral injection of a replication-defective type 5 adenovirus vector, AD5CMV-p53, to deliver the normal human p53 tumor suppressor gene.  The E1 region of AD5CMV-p53 has been replaced with a p53 expression cassette containing the human cytomegalovirus promoter (CMV).  Subjects will be divided into 2 treatment groups: (1) those with non-resectable tumors, and (2) those with surgically accessible tumors.  Subjects will receive multiple injections of vector in each dose-escalation cohort.  Following vector administration, subjects will be isolated for 48 hours during which time, assays will be conducted to demonstrate the lack of shedding of adenovirus vector.  The objectives of the study are to determine: (1) the maximum tolerated dose of AD5CMV-p53, (2) qualitative and quantitative toxicity related to vector administration, and (3) biologic activity. 

 

Prior to administration, adenovirus vector stocks will be screened for p53 mutants using the SAOS osteosarcoma cell assay that was submitted by Dr. Roth on June 23, 1995.  This biologic assay compares the activity of a standard stock of Adp53 vector to the activity of newly produced stocks.  The standard stock of Adp53 will be defined as mediating cell death in 100% of SAOS cells (human osteosarcoma cell line with homozygous p53 deletion) at an MOI of 50:1 (titer > 5x1010) on day 5 of culture.  The sensitivity of the assay for detecting inactive (presumed mutant) Adp53 vector will be determined by adding increasing amounts of Adluc to the Adp53 stock to determine the percentage of inactive vector required to decrease growth inhibition of SAOS cells mediated by Adp53.  The test lot of Adp53 will be tested for its ability to inhibit SAOS in a 5 day assay.  Significant loss of inhibitory activity compared with the standard would indicate an unacceptable level of inactive (presumed mutant) vector.  (Protocol #9412-096) 

 

Appendix D-107B.  Drs. Bernard A. Fox and Walter J. Urba of Earle A. Chiles Research Institute, Providence Medical Center, Portland, Oregon, may conduct gene transfer experiments on 18 subjects (18 years of age) with metastatic renal cell carcinoma or melanoma.  Autologous tumor cells will be surgically removed, transduced in vitro with the cationic liposome plasmid vector, VCL-1005, which encodes human leukocyte antigen (HLA)-B7 and beta-2 microglobulin.  Subjects will receive subcutaneous injection of lethally irradiated transduced cells in one limb.  The contralateral limb will be injected with lethally irradiated untransduced tumor cells in combination with Bacille Calmette-Guerin (BCG).  Approximately 21 days following tumor cell injection, subjects will undergo lymphadenectomy for subsequent in vitro expansion of anti-CD3 activated lymphocytes.  Activated lymphocytes will be adoptively transferred on approximately day 35 in combination with a 5-day course of interleukin-2 (IL-2).  On approximately day 45, subjects will receive a second cycle of IL-2.  The objectives of this study are to determine:  (1) the safety of administering anti-CD3 activated antitumor effector T cells in draining lymph nodes, and (2) whether HLA-B7/β-2 gene transfer augments the sensitization of anti-tumor effector T-cells in draining lymph nodes.  (Protocol 9506-108)

 

Appendix D-108.  Dr. Mitchell S. Steiner, University of Tennessee, Memphis, Tennessee, and Dr. Jeffrey T. Holt, Vanderbilt University School of Medicine, Nashville, Tennessee, may conduct gene transfer experiments on 15 male subjects (35 to 75 years of age) with metastatic prostate cancer.  Malignant cells obtained from advanced prostate cancer subjects have been demonstrated to express high levels of the protooncogene c-myc in vivo.  The mouse mammary tumor virus (MMTV) long terminal repeat (LTR) is expressed at high levels in prostate tissue.  Following removal of malignant cells via biopsy, subjects will receive a single transrectal ultrasound-guided intraprostate quadrant injection of the retrovirus vector, XM6:MMTV-antisense c-myc, for 4 consecutive days at the site of the original biopsy.  The objectives of this Phase I study are to:  (1) quantitatively assess the uptake and expression of XM6:MMTV-antisense c-myc by prostate cancer cells in vivo, (2) determine whether c-myc gene expression is prostate tumor-specific, (3) assess safety of intraprostate injection of XM6:MMTV-antisense c-myc, and (4) biologic efficacy (antisense inhibition of tumor growth).  (Protocol #9509-123)

 

Appendix D-109.  Drs. Ronald G. Crystal, Edward Hershowitz, and Michael Lieberman, New York Hospital-Cornell Medical Center, New York, New York, may conduct gene transfer experiments on 18 subjects (18 to 70 years of age) with metastatic colon carcinoma with liver metastases.  In this Phase I dose-escalation study, subjects will receive computed tomography (CT)-guided intratumoral injections of the adenovirus vector, AdGVCD.10, into the same hepatic metastasis in 4 equal volumes (100 microliters), each with a separate entry into the liver.  This dosage schedule will be performed on Days 1 and 7.  5-fluorocytosine (200 milligrams/ kilogram/24 hours) will be administered orally in 4 equal doses starting on day 2 and continuing through the time of laparotomy.  The objectives of this study are to:  (1) determine the dose-dependent toxicity of direct

 

administration of AdGVCD.10 to hepatic metastases combined with oral administration of 5-fluorocytosine, (2) quantitatively assess transfer and expression of the cytosine deaminase gene in target cells, and (3) determine the biologic effects of direct ADGVCD.10 administration to hepatic metastases. (Protocol #9509-125)

 

Appendix D-110.  Drs. Andres Berchuck and H. Kim Lyerly of Duke University Medical Center, Durham, North Carolina, may conduct gene transfer experiments on 18 subjects (18 years of age) with refractory metastatic ovarian cancer.  Autologous tumor cells obtained from ascites or surgically removed tumor will be transduced with the cationic liposome vector, PMP6A-IL2, that contains an adeno-associated virus derived plasmid DNA, a cytomegalovirus (CMV) promoter, and interleukin-2 (IL-2) complementary DNA (cDNA).  In this dose-escalation study, subjects will undergo 4 cycles of intradermal injections (thigh or abdomen) of ex vivo transduced, lethally irradiated tumor cells in an attempt to induce an antitumor response.  The objectives of the study are to evaluate:  (1) the safety of intradermally injected transduced cells, and (2) antitumor response following therapy. (Protocol #9506-110)

 

Appendix D-111.  Drs. Stephen L. Eck and Jane B. Alavi of the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania, may conduct gene transfer experiments on 18 subjects (>18 years of age) with malignant glioma.  The adenovirus vector encoding the Herpes simplex virus thymidine kinase (HSV-TK) gene, H5.020RSVTK, will be injected by a stereotactic guided technique into brain tumors.  Afterwards, the patients will receive systemic ganciclovir (GCV) treatment.  Patients eligible to undergo a palliative debulking procedure will receive the same treatment followed by resection on day 7, and a second dose of the vector intra-operatively.  Brain tissues removed by resection will be analyzed for adenovirus infection, transgene expression, and signs of inflammation.  The size and metabolic activity of tumors will be monitored by scanning with magnetic resonance imaging and positron emission tomography.  The objective of the study is to evaluate the overall safety of this treatment and to gain insight into the parameters that may limit the general applicability of this approach.  (Protocol #9409-089) 

 

Appendix D-112.  Drs. Robert Grossman and Savio Woo of the Baylor College of Medicine & Methodist Hospital, Houston, Texas, may conduct gene transfer experiments on 20 subjects (18 years of age) with refractive central nervous system malignancies.  Subjects will receive stereotaxic injections of a replication-defective, type 5 E1/E3-deleted adenovirus vector, ADV/RSV-tk, to deliver the Herpes simplex virus thymidine kinase (HSV-TK) gene to tumor cells.  Expression of the HSV-TK gene is driven by a Rous sarcoma virus long terminal repeat (RSV-LTR).  Subjects will receive a single time-course of intravenous ganciclovir (GCV) (14 consecutive days) following vector administration.  Following demonstration of safety with the initial starting dose of 1 x 108 particles in 5 subjects, additional cohorts will receive between 5 x 108 and 1.5 x 109 particles.  Each cohort will be monitored for toxicity for one month before administration of the next higher dose to subsequent cohorts.  Subjects will be monitored for evidence of clinical efficacy by magnetic resonance imaging and/or computer tomography scans.  The primary objective of this Phase I study is to determine the safety of vector administration.  (Protocol #9412-098)

 

Appendix D-113.  Drs. Gabriel N. Hortobagyi, Gabriel Lopez-Berestein, and Mien-Chie Hung, of the University of Texas MD Anderson Cancer Center, Houston, Texas, may conduct gene transfer experiments on a maximum of 24 adult patients (12 for each cancer) with metastatic breast or ovarian carcinoma.  Overexpression of the HER-2/neu oncogene occurs in 30% of ovarian and breast cancers, and it is associated with enhanced metastatic potential, drug resistance, and poor survival.  The E1A gene of the adenovirus type 5 functions as a tumor suppressor gene when transfected into cancer cells which overexpress the HER-2/neu oncogene.  E1A expression induces down regulation of the level of the HER-2/neu oncoprotein by a transcriptional control mechanism.  A plasmid, pE1A, encoding the adenovirus E1A gene with its own promoter will be administered as a DNA/lipid complex via the intraperitoneal or intrapleural route.  The objectives of the study are:  (1) to determine E1A gene transduction into malignant cells after the administration of E1A/lipid complex by intrapleural or intraperitoneal administration, (2) to determine whether E1A gene therapy can down-regulate HER-2/neu expression after intrapleural or intraperitoneal administration, (3) to determine the maximum biologically active dose (MBAD), or the maximum tolerated dose (MTD) of E1A/lipid complex, (4) to determine the toxicity and tolerance of E1A/lipid complex administered into the pleural or peritoneal space, and to assess the reversibility of such toxicity, and (5) to evaluate tumor response.  (Protocol #9512-137)

 

Appendix D-114.  Drs. Keith L. Black and Habib Fakhrai of the University of California, Los Angeles, California, may conduct gene transfer experiments on 12 subjects (18 years of age) with glioblastoma multiform.  An Epstein-Barr virus (EBV) based plasmid vector, pCEP-4/TGF- β2 antisense, encoding antisense RNA will be used to inhibit TGF- β2 production.  Tumor samples obtained from the patients at the time of clinically indicated surgery will be grown in culture to establish a cell line for each patient.  The patients' tumor cells will be genetically altered with the pCEP-4/TGF-β2 vector to inhibit their secretion of TGF-β.  Following completion of the traditional post surgical radiation therapy, the first cohort of patients will receive, at 3 week intervals, 4 injections of 5 x 106 irradiated gene modified autologous tumor cells.  Subsequently, in dose escalation studies, the second cohort will receive 1 x 107 cells, and the third cohort, 2 x 107 cells.  The results of this Phase I trial will be used to assess the safety of this form of gene therapy and may provide preliminary data to evaluate the potential utility of TGF- β2 antisense gene therapy in the management of gliomas.  (Protocol #9512-138)

 

Appendix D-115.  Dr. Ronald G. Crystal of New York Hospital-Cornell Medical Center, New York, New York, may conduct gene transfer experiments on a total of 21 (with an option for an additional 5) normal males and female subjects, age 18 years.  Replication-deficient adenovirus (Ad) vector previously has been used in a number of human gene therapy strategies to transfer genes in vivo for therapeutic purposes.  The purpose of this protocol is to characterize the local (skin), systemic (blood), and distant compartment (lung) immunity in normal individuals after intradermal administration of a replication deficient Ad5-based vector, named AdGVCD.10, carrying the gene coding for the E. coli enzyme, cytosine deaminase (CD).  Following intradermal administration of the vector to normal individuals, the skin, blood, and lung immune responses to the Ad vector and CD transgene will be evaluated over time.  This vector has been safety administered intrahepatically ten times to five individuals with colon carcinoma.  No adverse effects in Protocol #9509-125 have been observed.  The present protocol will yield insights into normal human immune responses to both the Ad vector, as well as to a heterologous (i.e., non-human) gene product (CD).  Note: This study is designed to answer basic biological questions regarding characterization of the immune responses to such vectors that have been previously documented.  (Protocol #9701-171)