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Your search term(s) "Aplastic Anemia or Myelodysplastic Syndromes" returned 42 results.
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ABC of Clinical Hematology. Williston, VT: Blackwell Publishing Inc. 2007. 99 p.
This book on clinical hematology is from a series of resource books written by specialists for nonspecialists. The book is designed to be easy to use and covers the symptoms, investigations, treatment, and management of conditions presenting in day-to-day practice. The book includes fifteen chapters covering iron deficiency anemia, macrocytic anemias, hereditary anemias, polycythemia, essential thrombocythemia and myelofibrosis, chronic myeloid leukemia, the acute leukemias, platelet disorders, the myelodysplastic syndromes, multiple myeloma and related conditions, bleeding disorders, thrombosis and anticoagulation, lymphoproliferative disorders including chronic lymphocytic leukemia, stem cell transplantation, hematological disorders at the extremes of life, hematological emergencies, and the impact of molecular biology and gene therapy on the field of hematology. The book includes full-color photographs and illustrations. Each chapter concludes with a list of references, and a detailed subject index appears at the end of the text.
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Aplastic Anemia: Pathogenesis and Treatment. IN: Hematology 2007. Washington, DC: American Society of Hematology.2007. pp 23-28.
This chapter, from Hematology 2007, highlights some of the contributions that have appeared in the literature in the past decade on the pathogenesis and treatment of aplastic anemia (AA). The author notes this field is large, including everything from stem cell biology to stem cell disorders, autoimmunity to transplantation, and graft-versus-host disease to late effects. The author discusses the immune pathogenesis of AA and notes that immunosuppressive therapy (IST) remains an important option for AA patients who are not candidates for transplantation. Favorable prognostic indicators for IST are young age and a short interval from diagnosis. Current antithymocyte globulin–cyclosporin combination therapy can result in a skewed neutrophil count that is no longer useful as a predictor. The outcome of allogeneic bone marrow transplantations has significantly improved in the past decade, particularly in the unrelated donor setting, to such an extent that treatment strategies may be affected. The author concludes by emphasizing the need of early referral to an experienced treatment facility for these patients, particularly because a short interval between diagnosis and treatment will improve results for bone marrow transplantation. 2 figures. 39 references.
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Bone Marrow Failure as a Risk Factor for Clonal Evolution: Prospects for Leukemia Prevention. IN: Hematology 2007. Washington, DC: American Society of Hematology. pp 40-46.
This chapter, from Hematology 2007, reviews the association of bone marrow failure and the development of leukemia. The authors note that patients with bone marrow failure syndromes are at risk for the development of clonal neoplasms, including paroxysmal nocturnal hemoglobinuria (PNH), myelodysplasia (MDS), and acute myelogenous leukemia (AML). Approximately 10 to 20 percent of those who survive acquired aplastic anemia will develop a clonal disease within the decade following their diagnosis. The relative risk of clonal neoplasms is very significantly increased in children and adults with inherited bone marrow failure syndromes as well. Recent research studies support a model in which cells resistant to extracellular apoptotic cues are selected from the stem cell pool. In the past 2 years this paradigm has been validated in preclinical models that are robust enough to reconsider new therapeutic objectives in aplastic states and to support the planning and development of rationally designed leukemia prevention trials in patients at increased risk. 2 figures. 46 references.
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Clinical Malignant Hematology. Columbus, OH: McGraw Hill. 2007. 1201 p.
This comprehensive reference book covers the full spectrum of cancers in the blood, bone marrow, and lymphatic system, including leukemia, lymphoma, and myeloma. The textbook includes 109 chapters in 15 sections: acute myeloid leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphocytic leukemia, hairy cell leukemia, special topics in leukemia, myelodysplastic syndromes, chronic myeloproliferative diseases, non-Hodgkin’s lymphoma, Hodgkin’s lymphoma, special topics in lymphoma, multiple myeloma, special topics in multiple myeloma, stem cell transplantation, and special topics in neoplastic hematology. Each chapter details the workup, staging, epidemiology, risk factors, pathology, symptoms, diagnosis, differential diagnosis, and treatment of hematologic malignancies. The chapters include full coverage of all treatment options such as chemotherapy, monoclonal antibodies, and hematopoietic stem cell transplantation. The text includes background chapters that offer guidance on how to address treatment complications and other supportive care issues. The textbook is illustrated with black-and-white clinical pictures and photomicrographs displaying examples of peripheral blood smears and bone marrow aspirates; one section of full-color plates is included. A detailed subject index concludes the volume.
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Diagnosis, Genetics, And Management of Inherited Bone Marrow Failure Syndromes. IN: Hematology 2007. Washington, DC: American Society of Hematology.2007. pp 29-39.
This chapter, from Hematology 2007, reviews the diagnosis, genetics, and management of inherited bone marrow failure syndromes. These syndromes are traditionally considered to be pediatric disorders, but many of the patients are now diagnosed as adults, and many diagnosed as children now live to reach adulthood. The most common of these rare disorders include Fanconi anemia, dyskeratosis congenita, Shwachman-Diamond syndrome and amegakaryocytic thrombocytopenia. Patients with these disorders may develop aplastic anemia or myelodysplastic syndrome and acute myeloid leukemia. Other inherited bone marrow failure syndromes include Diamond-Blackfan anemia, severe congenital neutropenia, and thrombocytopenia absent radii; these are single cytopenias that rarely if ever become aplastic but carry increased risks of leukemia. In addition, the first three syndromes are associated with high risks of solid tumors: head and neck and anogenital squamous cell carcinoma in Fanconi anemia and dyskeratosis congenita, and osteogenic sarcoma in Diamond-Blackfan anemia. The diagnosis of a marrow failure syndrome requires recognition of characteristic physical abnormalities and a suspicion for these disorders in the differential diagnosis of patients who present with acquired aplastic anemia, myelodysplastic syndrome, acute myeloid leukemia, or atypically early cancers of the types seen in the syndromes. The author concludes that better diagnosis and proof of etiology will come from the identification of pathogenic mutations in genes associated with each syndrome. 4 tables. 38 references.
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Etiology And Management of Therapy-Related Myeloid Leukemia. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 453-459.
This chapter reviews the etiology and management of therapy-related myeloid leukemia (t-AML) and therapy-related myelodysplastic syndromes (t-MDS). When these diseases are identified, they denote a group of high-risk patients with multiple and varied poor prognostic features. These neoplasms are thought to be the direct consequence of mutational events caused by cytotoxic cancer therapy. The author considers whether a diagnosis of t-AML per se indicates a poor prognosis or whether the bad outcomes seen in these patients result from other clinical and biologic characteristics. Because of lingering damage from prior cytotoxic therapy and, in some cases, the persistence of their primary disorder, patients with t-AML are often poor candidates for intensive AML therapy. The frequency of unfavorable cytogenetics, such as a complex karyotype or deletion or loss of chromosomes 5 and/or 7, is higher in t-AML. Survival varies according to cytogenetic risk group, with better outcomes observed in patients with t-AML with favorable-risk karyotypes. The author stresses that treatment recommendations should be based on performance status and karyotype. Patients with t-AML should be included in front-line chemotherapy trials, appropriate for de novo AML patients with similar disease characteristics. Allogeneic hematopoietic cell transplantation can cure some patients with t-AML.
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Genetic Pathways in the Pathogenesis of Therapy-Related Myelodysplasia and Acute Myeloid Leukemia. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 392-397.
This article, from the Hematology 2007 monograph, reviews the genetic pathways relevant to the pathogenesis of therapy-related myelodysplasia (t-MDS) and acute myeloid leukemia (t-AML). The authors note that in these conditions, at least eight alternative genetic pathways have been defined based on characteristic recurrent chromosome abnormalities. The cytogenetic pattern in the patients depends on the type of leukemogenic therapy received: alkylating agents, topoisomerase II inhibitors, or radiotherapy. The authors describe the three types of gene mutations that are observed in MDS and AML: activating mutations of genes in the tyrosine kinase–RAS/BRAF signal transduction pathway, leading to increased cell proliferation (Class I mutations); inactivating mutations of genes encoding hematopoietic transcription factors, resulting in disturbed cell differentiation (Class II mutations); and inactivating mutations of the tumor suppressor gene p53. Class I and Class II mutations are significantly associated, indicating their cooperation in leukemogenesis. The authors conclude that therapy-related and de novo MDS and AML are identical diseases in terms of cytogenetic abnormalities and gene mutations and should be subclassified and treated similarly. 2 tables. 44 references.
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Implication of the Molecular Characterization of Acute Myeloid Leucemia. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 412-419.
This chapter, from the Hematology 2007 monograph, reviews the current understanding of the molecular characterization of acute myeloid leukemia (AML). The author notes that the recent identification of molecular genetic alterations such as gene mutations or deregulated gene expression in AML has greatly advanced the understanding of leukemogenesis. These markers now permit some classification of the enormous heterogeneity seen within cytogenetically defined subgroups of AML. Furthermore, the molecular alterations are providing targets for molecular therapies. In this chapter, the author reviews major molecular findings of prognostic and predictive significance, with an emphasis on the discussion of gene mutations found in two major AML subgroups: cytogenetically normal and core-binding factor AML. The author reports on the markers that are about to enter clinical practice, most in the context of clinical trials. 2 figures. 1 table. 52 references.
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Management of RBC-Transfusion Dependence. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 398-404.
This chapter, from the Hematology 2007 monograph, reviews the management of patients with red blood cell (RBC)-transfusion dependence. The authors note that strategies for the management of anemia in patients with myelodysplastic syndrome (MDS) include the use of three new therapeutics. These treatments range from symptom amelioration with RBC transfusions to more active treatment of the disease. However, most patients develop transfusion-dependent anemia over the course of their disease, and its adverse consequence on the natural history of disease has only recently been appreciated. Transfusion dependence increases the risk of organ complications from iron overload coupled with an increased risk of leukemia transformation. Anemia severity is an important variable limiting the otherwise favorable natural history of patients with lower risk disease. The authors focus on therapeutic strategies that successfully restore effective erythropoiesis, such as erythropoietic stimulating agents, immunomodulatory agents, immunosuppressive therapies, or hypomethylating agents. These treatments may favorably affect the natural history of this disease, creating perhaps a new urgency for the initiation of erythropoietic promoters that have durable clinical benefit. The authors conclude with a brief discussion of the selection of primary therapy for the management of anemia, including the importance of considering four factors: age, RBC transfusion burden and duration, endogenous erythropoietin production, and karyotype. 2 tables. 40 references.
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Modifying the Epigenome as a Therapeutic Strategy in Myelodysplasia. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 405-411.
This chapter, from the Hematology 2007 monograph, describes strategies to modify the epigenome as a treatment approach in myelodysplasia. The author defines epigenetics as a number of biochemical modifications of chromatin that, without altering the primary sequence of DNA, have a role in genomic regulation and in particular gene expression control. Clinical interest in these epigenetic alterations stems mainly from two observations. First, detection of specific epigenetic alterations could be used to develop cancer biomarkers (e.g., for the early detection or prognostication of cancer). Second, most epigenetic alterations are reversible both in vitro and in vivo, leading the way to the development of new anti-cancer therapies. The author provides a review of the current clinical information regarding different forms of epigenetic therapy in patients with myelodysplastic syndromes (MDS). Two DNA hypomethylating agents are currently approved for patients with MDS: 5-azacitidine and 5-aza-2-dioxycitidine. 5-azacitidine has been associated with an increased response rate and time to AML transformation, as well as a trend toward improved overall survival and improved quality of life, compared with supportive care. Basic aspects of DNA methylation or histone code alterations are not covered in detail in this review. The author concludes by calling for additional research in three areas: the discovery of predictive biomarkers of response; the development of newer DNA hypomethylating agents; and the development of new alternative forms of epigenetic modifiers. 1 figure. 2 tables. 43 references.
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Myelodysplastic Syndromes. IN: Sekeres, M.; Kalaycio, M.; Bolwell, B., eds. Clinical Malignant Hematology. Columbus, OH: McGraw Hill. 2007. pp 367-444.
This section about myelodysplastic syndromes (MDS) is from a comprehensive reference book that covers the full spectrum of cancers in the blood, bone marrow, and lymphatic system, including leukemia, lymphoma, and myeloma. The myelodysplastic syndrome is a heterogeneous group of clonal neoplastic stem cell disorders and is characterized by bone marrow failure with peripheral cytopenias and a tendency to progress to acute myeloid leukemia (AML). This section offers seven chapters: molecular biology, pathology, and cytogenetics in MDS; clinical features and making the diagnosis; the recommended treatment approach for early MDS; aplastic anemia and stem cell failure; allogeneic stem cell transplantation for MDS and aplastic anemia; definition of remission, prognosis, and follow-up in patients with MDS; and treatment of relapsed or refractory MDS and new frontiers in MDS therapy. The chapters are illustrated with black-and-white clinical pictures and photomicrographs. Each chapter concludes with an extensive list of references.
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Myelodysplastic Syndromes. IN: Provan, D., ed. ABC of Clinical Haematology. Williston, VT: Blackwell Publishing Inc. 2007. pp 40-44.
The myelodysplastic syndromes (MDS) are a group of clonal hemopoietic disorders characterized by ineffective hemopoiesis resulting in anemia and an increased risk of transformation to acute myeloid leukemia (AML). This chapter about MDS is from a book on clinical hematology, written by specialists for nonspecialists. The book is designed to be easy to use and covers the symptoms, investigations, treatment, and management of conditions presenting in day-to-day practice. In this chapter, the authors review etiology and pathogenesis, diagnosis, recommended investigations, classification, prognosis, and patient care management. The authors note that MDS is primarily a disease of the elderly, with a median age at diagnosis of 60 to 75 years. Treatment ranges from supportive care with blood products, immunotherapy, and chemotherapy, to allogeneic bone marrow transplantation, depending on the age and fitness of the patient and the severity of the myelodysplasia. New drug therapies, including lenalidomide and azacytidine, are in the clinical trial stages of development. The chapter is illustrated with full-color photographs, drawings, and charts. 4 figures. 4 tables. 6 references.
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Nutritional Concerns for Individuals with Hematological Disorders. Aplastic Anemia & MDS International Foundation Newsletter. 20(1): 2, 9. Winter 2007.
This newsletter article helps readers with hematological disorders understand some of the nutritional issues that may be of concern to them. The authors discuss diet, food and medication interactions, and the use of supplements for people with bone marrow disease. The authors stress that proper nutrition optimizes blood production. Dietary modifications for people with aplastic anemia, myeloproliferative disorders, and PNH are intended to minimize the introduction of pathogenic organisms from food. Readers are encouraged to work closely in tandem with their health care providers to incorporate any dietary changes or the use of supplements.
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Philadelphia Chromosome–Positive Acute Lymphocytic Leukemia: A New Era of Challenges. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 435-443.
This chapter reviews significant advances in the treatment of Philadelphia chromosome positive (PH+) or BCR-ABL–positive acute lymphocytic leukemia (ALL) that have been made since the discovery of the selective ABL tyrosine kinase inhibitors (TKIs). The author notes that the outcome in these patients with standard chemotherapy was previously dismal, but incorporation of imatinib mesylate into frontline therapy has improved relapse-free and overall survival. The benefits of imatinib are particularly apparent in patients where allogeneic stem cell transplantation in first complete remission is prohibited by older age, comorbidities, or lack of a suitable donor. However, the emergence of resistance to imatinib presents new therapeutic challenges. The development of novel TKIs with enhanced inhibitory potency against ABL and other kinases may further improve on the results observed with imatinib. Optimal use of these novel agents in the treatment schema of Ph+ ALL will be paramount in ensuring continued success in the eradication of this disease. The author reviews the details of patient care management of this population. 2 figures. 2 tables. 66 references.
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Targeting Treatment in AML. IN: Hematology 2007. Washington, DC: American Society of Hematology. 2007. pp 429-434.
This chapter about targeting treatments in acute myeloid leukemia (AML) is from the Hematology 2007 monograph. The authors stress that the currently available chemotherapy has probably reached the limits of its potential in treating AML. They describe some of the next steps in the evolution of treatment for AML, which need to exploit knowledge of the molecular, immunophenotypic, and biological characteristics of the disease on the one hand and, on the other, the biology of the patient. Immunophenotyping has defined an adequate target (CD33) for antibody-directed treatment, although this is not leukemia specific. In most patients a leukemia-specific immunophenotype can be characterized that can be used to monitor treatment. Minimal residual disease (MRD) detection in morphological remission can detect patients at high risk of relapse, as can a limited number of molecular markers. The clinical value of intervening at the time of MRD detection is not clear. Among the increasing molecular abnormalities described in AML, FLT-3 mutations appear the most attractive for therapeutic intervention. Other mechanisms that can be specifically targeted include farnesylation, methylation status, and histone deacelylation. The authors discuss newer treatments that exploit characteristics of the leukemic stem cells that differ from the normal stem cell. 2 tables. 40 references.
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Which Patients with Adult Acute Lymphoblastic Leukemia Should Undergo a Hematopoietic Stem Cell Transplantation? Case-Based Discussion. IN: Hematology 2007.Washington, DC: American Society of Hematology. 2007. pp 444-452.
This chapter, from the Hematology 2007 monograph, considers the factors involved in the decision to proceed to hematopoietic stem cell transplant (HSCT) for adult patients with acute lymphoblastic leukemia (ALL). The authors note that relapse and nonrelapse mortality continue to plague the outcome of HSCT even when undertaken in complete remission (CR). Those considered to be at high risk for relapse often are considered for HSCT in first complete remission (CR1), whereas those at lower risk may not be referred until they have relapsed, when their chances for cure are very poor. In some patients who have a suitable histocompatible sibling, disease- or patient-related factors may override the potential benefit of allogeneic HSCT. Because many patients do not have a suitable histocompatible sibling, one has to consider the relative merits of autologous transplantation versus use of an alternative allogeneic stem cell source such as a matched-unrelated donor (MUD), umbilical cord blood (UCB) donor, or haploidentical donor. Deciding among these options versus chemotherapy—even in high-risk patients—is difficult. In the review, the authors discuss the risks and benefits of these choices to determine whether and by what means to proceed to HSCT in adult patients with ALL who are in CR1. The authors present two case studies of patients with ALL and a discussion of how the data provided would lead to a decision about the selection of therapy. 1 figure. 3 tables. 52 references.
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Yin And Yang of Stem Cell Gene Therapy: Insights into Hematopoiesis, Leukemogenesis, And Gene Therapy Safety. IN: Hematology 2007. Washington, DC: American Society of Hematology. 2007. pp 460-465.
This chapter, from the Hematology 2007 monograph, describes stem cell gene therapy, focusing on hematopoiesis, leukemogenesis, and gene therapy safety. Topics include the use of replication-incompetent retroviral vectors to modify hematopoietic stem cells (HSCs), adverse events related to these vector insertions, the genotoxicity of integrating retroviruses, and approaches to decreasing genotoxic risk. Diseases for which these techniques may be appropriate include HIV and leukemia; much of the research discussed consists of animal studies. The ultimate goal of these types of therapies is correction of a defective gene via homologous recombination. The author calls for continued investigation of vector biology; genomic, viral, and cellular determinants of integration sites; and target cell characteristics. 1 table. 47 references.
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Aplastic Anemia and Bone Marrow Failure. IN: Hoffbrand, A.V.; Moss, P.A.H.; Pettit, J.E. Essential Haematology. 5th ed. Williston, VT: Blackwell Publishing Inc. 2006. pp. 241-248.
This chapter on aplastic anemia and bone marrow failure is from a hematology textbook that offers a comprehensive look at the biochemical, physiological, and immunological processes involved in normal blood cell formation and function and the disturbances that may occur in different diseases. The authors discuss pancytopenia, aplastic anemia, red cell aplasia, Schwachman-Diamond syndrome, and congenital dyserythropoietic anemias (CDAs). Pancytopenia is a reduction in the blood count of all the major cell lines: red cells, white cells, and platelets. Aplastic, or hypoplastic, anemia is defined as pancytopenia resulting from aplasia of the bone marrow. Fanconi’s anemia is a congenital type of aplastic anemia. Red cell aplasia is a rare syndrome characterized by anemia with normal leucocytes and platelets and grossly reduced or absent erythroblasts in the marrow. Schwachman-Diamond syndrome is a rare autosomal recessive syndrome, characterized by varying degrees of cytopenia. CDAs are a group of hereditary refractory anemias characterized by ineffective erythropoiesis and erythroblast multinuclearity. The chapter features full-color photographs and illustrations. 4 figures. 3 tables. 14 references.
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Bone Marrow Diseases Explained: Aplastic Anemia, Myelodysplastic Syndromes (MDS) and Paroxysmal Nocturnal Hemoglobinuria (PNH). Annapolis, MD: Aplastic Anemia and MDS International Foundation. 2006. 4 p.
Bone marrow diseases occur when the bone marrow stops making enough healthy blood-forming stem cells. Blood-forming stem cells act like factories to produce the body's three types of blood cells: red, white, and platelets. This brochure reviews three types of bone marrow diseases: aplastic anemia, myelodysplastic syndromes (MDS), and paroxysmal nocturnal hemoglobinuria (PNH). Aplastic anemia occurs when the bone marrow stops making enough stems cells, which reduces the number of platelets and red and white blood cells. MDS occurs when bone marrow produces defective stem cells, which results in lower blood counts for one or more types of blood cells, and the cells that are produced tend to be immature or defective. PNH occurs when defective stem cells create red blood cells that have a defective protective layer, resulting in the cells being destroyed by the body's immune system. The brochure encourages readers to educate themselves about these conditions so they can be an active part of their own health care team. A brief glossary of relevant terms is included, as is a list of questions to ask one's health care provider. The brochure emphasizes that because these are such rare diseases, it is vital that patients receive up-to-date medical information, expert treatment, and emotional support.
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Bone Marrow Failure. IN: Nilsson, K.R.; Piccini, J.P., eds. Osler Medical Handbook. Philadelphia, PA: Saunders. 2006. pp. 510-515.
This chapter on bone marrow failure is from a handbook that provides the essentials of diagnosis and treatment, as well as the latest in evidence-based medicine, for residents working bedside, in-patient care. The chapter begins with a presentation of essential Fast Facts and concludes with Pearls and Pitfalls useful to the practicing internist. The body of the chapter is divided into sections: Epidemiology, Clinical Presentation, Diagnosis, and Management. Specific topics covered in this chapter include aplastic anemia, a disorder of bone marrow failure characterized by peripheral pancytopenia and a hypocellular bone marrow; pancytopenia, a deficiency of all cellular blood elements (anemia, neutropenia, and thrombocytopenia); acquired and congenital aplastic anemia; the presenting symptoms of pancytopenia, including fatigue, dyspnea on exertion, fever, easy bruising, and increased susceptibility to infections; and the use of bone marrow examination in patients with aplastic anemia. The chapter concludes with a list of references, each labeled with a 'strength of evidence' grade to help readers determine the type of research available in that reference source. 2 figures. 11 references.
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Classification, Diagnosis and Management of Myeloproliferative Disorders in the JAK2V617F Era. Washington, DC: American Society of Hematology. 2006. pp. 240-245.
This article reviews the classification, diagnosis, and management of myeloproliferative disorders now that JAK2V617F, has been identified. JAK2V617F , a somatic gain-of-function mutation involving the JAK2 tyrosine kinase gene, occurs in nearly all patients with polycythemia vera (PV) but also in a variable proportion of patients with other myeloid disorders. The mutation frequency is estimated at approximately 50 percent in both essential thrombocythemia (ET) and myelofibrosis (MF), up to 20 percent in certain subcategories of atypical myeloproliferative disorder (atypical MPD), less than 3 percent in de novo myelodysplastic syndrome (MDS) or acute myeloid leukemia, and 0 percent in chronic myeloid leukemia (CML). The author presents the case for grouping PV, ET, and MF together in a distinct MPD category that is separate from chronic myeloid leukemia (CML), MDS, and atypical MPD. The author concludes that the presence of JAK2V617F strongly suggests an underlying MPD and it is therefore reasonable to consider JAK2V617F-based laboratory tests for the evaluation of polycythemia, primary thrombocytosis, unexplained leukocytosis, bone marrow fibrosis, or abdominal vein thrombosis. A patient care algorithm is also included. 1 figure. 3 tables. 41 references.
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Clinical Issues in the Management of Patients with Myelodysplasia. IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 205-210.
This article reviews the management of patients with myelodysplasia (MDS), which can be quite complex and which varies according to both the clinical manifestations in individual patients as well as complicating medical conditions. Allogeneic stem cell transplantation is the only curative treatment; however, in the older patient population it must be applied selectively, particularly in those with lower risk MDS as well as in patients whose clinical course is more frankly preleukemic. The author discusses issues pertinent to the use of 5-azacytidine, decitabine and lenalidomide in patients with both higher and lower International Prognostic Staging System (IPSS) stage disease. The author concludes by calling for innovative clinical trials to address the lack of satisfactory treatments for this patient population. Meanwhile, the best management of MDS requires attention from experienced physicians to provide reduction of symptoms and maximal extension of life. 31 references.
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Essential Haematology. 5th ed. Williston, VT: Blackwell Publishing Inc. 2006. 380 p.
This hematology textbook offers a comprehensive look at the biochemical, physiological, and immunological processes involved in normal blood cell formation and function and the disturbances that may occur in different diseases. The book is designed to help medical students grasp the essential features of modern clinical and laboratory hematology. The book includes 28 chapters on hemopoiesis, erythropoiesis and general aspects of anemia, hypochromic anemias and iron overload, megaloblastic anemias and other macrocytic anemias, hemolytic anemias, genetic disorders of hemoglobin, granulocytes and monocytes, lymphocytes and their benign disorders, the spleen, the etiology and genetics of hematological malignancies, the management of hematological malignancy, acute leukemias, chronic myeloid leukemia, myelodysplasia, the chronic lymphoid leukemias, Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, multiple myeloma and related disorders, myeloproliferative disorders, aplastic anemia and bone marrow failure, stem cell transplantation, platelets and blood coagulation, bleeding disorders caused by vascular and platelet abnormalities, coagulation disorders, thrombosis and antithrombotic therapy, hematological changes in systemic disease, blood transfusion, and pregnancy and neonatal hematology. Each chapter includes full-color photographs and illustrations and concludes with a list of references. The book concludes with three appendices and a detailed subject index.
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Hematopathological Concepts and Controversies in the Diagnosis and Classification of Myelodysplastic Syndromes. IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 199-204.
Although the diagnosis and classification of most cases of the myelodysplastic syndromes (MDS) are usually accomplished without difficulty, a minority of cases may pose diagnostic problems. This article explores hematopathological concepts and controversies in the diagnosis and classification of MDS. The author contends that, in many cases, the diagnostic dilemma can be solved by adhering to basic guidelines recommended for evaluation of patients suspected of having MDS, and in particular to the quality of the blood and bone marrow specimens submitted for morphologic, immunophenotypic and genetic studies. In other cases, such as patients who have hypocellular MDS or MDS with fibrosis, the criteria for making a diagnosis may be difficult if not impossible to apply. The author presents data to illustrate that the World Health Organization (WHO) classification offers a valuable tool in the diagnosis and classification of MDS. 1 table. 34 references.
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Inherited Bone Marrow Failure Syndromes: Molecular Features. IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 63-71.
This article reviews recent advances resulting from the identification of the genes responsible for four inherited marrow failure syndromes: Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, and Shwachman-Diamond syndrome. The inherited marrow failure syndromes are characterized by impaired hematopoiesis and cancer predisposition, as well as a range of congenital anomalies. The author stresses that interpretation of the genetic testing should be guided by an understanding of the limitations of such testing for each disorder. The possibility of an inherited basis for marrow failure must be considered for adults as well as children with aplastic anemia. Shared molecular themes are emerging from functional studies of the genes underlying the different inherited disorders. Genomic instability may result from impaired DNA repair in Fanconi anemia or telomere dysregulation in dyskeratosis congenita. Mutations affecting ribosome assembly or function are associated with Diamond-Blackfan anemia, dyskeratosis congenita, and Shwachman-Diamond syndrome. The author focuses on the clinical implications arising from these molecular studies. 4 figures. 2 tables. 46 references.
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Making Therapeutic Decisions in Adults with Aplastic Anemia. IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 78-85.
This article reviews the therapeutic management of adults with aplastic anemia (AA). The author recommends careful exclusion of other causes of bone marrow failure and cautions that late-onset inherited forms of AA may present in adulthood with subclinical disease. Recent long-term studies of HLA-identical sibling donor bone marrow transplantation (BMT) show excellent survival for patients under the age of 40 years, but chronic graft-versus-host disease (GVHD) is still a major problem, particularly for quality of life. Recent improvements in outcome after matched, unrelated-donor BMT may reflect better donor matching and use of reduced intensity conditioning regimens. For patients treated with immunosuppressive therapy, antithymocyte globulin (ATG) and cyclosporin (CSA) remain the standard regimen with excellent overall survival but less impressive failure-free survival due to nonresponse, relapse, and later clonal disorders. The benefit of adding granulocyte colony-stimulating factor (G-CSF) to ATG and CSA is unclear and being assessed in a further prospective European study. The author notes that patients who are refractory to conventional immunosuppressive therapy and currently ineligible for BMT represent difficult management problems. For this patient population, new approaches to transplantation are being evaluated, such as fludarabine-based conditioning regimens and the potential use of double umbilical cord blood transplants, but there is a need for new immunosuppressive agents. The author concludes that improved supportive care is likely to be a major factor in better outcome for all AA patients regardless of treatment approach. 1 figure. 2 tables. 40 references.
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Myelodysplasia. IN: Hoffbrand, A.V.; Moss, P.A.H.; Pettit, J.E. Essential Haematology. 5th ed. Williston, VT: Blackwell Publishing Inc. 2006. pp. 182-187.
Myelodysplastic syndromes are disorders of multipotent hemopoietic stem cells that are characterized by increasing bone marrow failure with quantitative and qualitative abnormalities of all three myeloid cell lines. Cytopenias often accompany a marrow of normal or increased cellularity. Patients have a tendency to progress to acute myeloid leukemia (AML), although death often occurs before this develops. This chapter on myelodysplasia is from a hematology textbook that offers a comprehensive look at the biochemical, physiological, and immunological processes involved in normal blood cell formation and function and the disturbances that may occur in different diseases. The authors describe the pathogenesis, clinical features, laboratory findings, classification, and treatment of myelodysplastic syndromes. A final section briefly reviews chronic myelomonocytic leukemia and juvenile myelomonocytic leukemia. The chapter includes full-color illustrations and photographs. 3 figures. 3 tables. 8 references.
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Myelodysplastic Syndromes. White Plains, NY: Leukemia and Lymphoma Society. 2006. 33 p.
The term myelodysplastic syndromes (MDS) has been used since the late 1970s to describe a group of blood cancers. In MDS, blood cell counts are usually low, but the decrease in counts of red cells, white cells, and platelets may occur to different degrees. MDS subtypes can vary from those that are nonprogressive and have little effect on a person’s health to those that may result in anemia, other problems that require treatment, or serious effects on health and life expectancy. This booklet provides information about MDS for patients and their families. The booklet first describes the normal blood and marrow and then reviews the incidence, causes and risk factors, range of severity, and diagnosis of MDS. Disease subtypes are outlined, including the chronic or nonprogressive forms of MDS, progressive and symptomatic forms of MDS, how the disease normally progresses, and the use of the International Prognostic Scoring System. Additional sections cover determining need for treatment and treatment approaches, supportive care, lower-intensity therapy, higher intensity therapy, clinical trials, and the social and emotional aspects of MDS. The booklet includes a glossary of terms and a list of resources for more information, as well as the contact information for the Leukemia and Lymphoma Society. 1 figure. 2 tables. 13 references.
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Pathophysiologic Mechanisms in Acquired Aplastic Anemia. IN: Hematology 2006. Washington, DC: American Society of Hematology. 2006. pp 72-77.
This article explores the pathophysiologic mechanisms in acquired aplastic anemia, an unusual hematologic disease characterized by an absence of hematopoietic cells. An immune pathophysiology has been inferred for this disease from improvement in blood counts with immunosuppressive therapy in the majority of patients. Molecular mechanisms underlying both T cell effector cells and the target marrow stem and progenitor cells are now being identified. Activated type 1 cytotoxic T cells and type 1 cytokines have been implicated in cell culture experiments; clues to the molecular basis of the aberrant immune response include cytokine gene polymorphisms and abnormalities in the regulatory pathways for gamma-interferon. For stem cell depletion, mutations in genes of the telomere repair complex are present in some patients with apparently acquired aplastic anemia. Telomerase deficiency is associated with short telomeres and a quantitative reduction in marrow progenitors and likely also a qualitative deficiency in the repair capacity of hematopoietic tissue. The author concludes that quantitative and practical measurements of oligoclonal T cell activity and of hematopoietic stem cell number and function may allow laboratory testing to guide treatment decisions. 42 references.
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Revlimid Approved by the FDA. Aplastic Anemia and MDS International Foundation, Inc. 19(1): 1. Winter 2006.
This brief newsletter article reports on the recent approval by the U.S. Food and Drug Administration (FDA) of the drug lenolidomide (Revlimid) for the treatment of patients with a subgroup of myelodysplastic syndromes (MDS). This oral medication is approved for the treatment of low- or intermediate-risk MDS associated with deletion of 5q, a chromosomal abnormality. Lenolidomide is an immunomodulatory drug, chemically similar to thalidomide. The article notes that because of concerns about severe birth defects occurring in any women who may become pregnant while on lenolidomide, or whose male partners may be taking lenolidomide, the drug is being marketed and supported by a special risk management program called RevAssist (www.revlimid.com or 800-747-2820).
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Treatment of the 5q-Syndrome. Washington, DC: American Society of Hematology. 2006. pp. 192-198.
This article reviews treatment of the 5q- syndrome, a specific type of myelodysplastic syndrome (MDS) that presents with particular characteristics, including severe anemia, frequent thrombocytosis, typical dysmegakaryopoiesis, and favorable outcome. The pathogenesis of the 5q- syndrome remains uncertain, in particular the role of inactivation of gene(s) situated in 5q. The authors note that, until the advent of lenalidomide, repeated red blood cell (RBC) transfusions were generally the only treatment of the 5q- syndrome, which was resistant to other therapeutic approaches. Lenalidomide can lead to RBC transfusion independence in at least two-thirds of cases of the 5q- syndrome, with two-thirds of those responses persisting after 2 years of treatment. Not only reversal of anemia but also frequent complete pathological and cytogenetic responses are obtained. The authors caution that Grade 3 or 4 neutropenia and thrombocytopenia, especially during the first 6 to 8 weeks of treatment, are the major side effect of lenalidomide, so patients should have close monitoring of blood counts and regular physician visits. 3 tables. 39 references.
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Aplastic Anemia: Management of Adult Patients. IN: Hematology 2005. Washington, DC: American Society of Hematology. 2005. pp. 110-117.
This article reviews the management of adult patients with acquired aplastic anemia (AA). The authors discuss the clinical features of AA in adults, diagnostic considerations, clinical subentities, conservative therapies, coping with relapse, salvage therapies, bone marrow transplantation (BMT), complications of transplantation, and prognosis. Immunosuppressive therapy with antithymocyte globulin (ATG) and cyclosporine (CsA) constitutes the primary treatment option and may be better than BMT in older patients. There are very few clinical clues as to the selection of patients who are likely to respond to immunosuppression. Conservative therapy such as intense immunosuppression is associated with a high relapse rate but does not impact the survival and overall prognosis. The inability to eliminate autoimmune T cell clones using current therapeutic strategies suggests prolonged immunosuppressive maintenance therapy may be needed for a substantial proportion of patients. Bone marrow transplantation is associated with several complications, including an increased frequency of solid tumors. The authors conclude that new immunosuppressive and immunomodulatory agents, as well as constantly improving results of allogeneic BMT, will further improve the survival rate of adult patients with AA. 1 figure. 5 tables. 40 references.
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Aplastic Anemia: Management of Pediatric Patients. IN: Hematology 2005. Washington, DC: American Society of Hematology. 2005. pp. 104-109.
This article reviews the management of pediatric patients with aplastic anemia, a condition that features pancytopenia and bone marrow hypoplasia arising from a variety of disease states. The author cautions that the management of children with these disorders has been complicated by difficulties of diagnosis. The availability of molecular testing has assisted in partial resolution of this problem but has raised new issues, such as the potential of genetic predisposition and the management of asymptomatic individuals with molecular markers. In addition, data from some large cohort studies and disease registries are providing a rational basis for making more informed treatment decisions for children with these disorders. Approaches to hematopoietic stem cell transplantation (SCT), using both conventional and alternative donors, are changing rapidly. The author recommends a multidisciplinary approach to managing both the underlying problems and the significant sequelae of treatment in both acquired and congenital disease. 46 references.
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Bone Marrow Failure: A Child Is Not Just a Small Adult (But an Adult Can Have a Childhood Disease). IN: Hematology 2005. Washington, DC: American Society of Hematology. 2005. pp. 96-103.
This article helps readers understand the differences between inherited bone marrow failure syndromes (IBMFS), typical in children, and acquired aplastic anemia, which is more associated with adults. The distinction between these two conditions is not in the age of the patient, but in the clinical and laboratory diagnoses. Physicians for adults must consider the inherited disorders as part of their diagnostic process and must also realize that children with inherited disorders now survive to make the transition to adult care. The author reviews the major inherited bone marrow failure syndromes associated with development of pancytopenia, including Fanconi anemia, dyskeratosis congenital, Shwachman-Diamond syndrome, and amegakaryocytic thrombocytopenia. The ages at presentation are highly variable but often include individuals of adult age who have previously undiagnosed Fanconi anemia or dyskeratosis congenita. Many of the genes responsible for these disorders have been identified. The author concludes by reiterating that the correct classification of patients with aplastic anemia of any age is required for an appropriate management plan. 1 figure. 3 tables. 44 references.
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Families Coping with Bone Marrow Failure Diseases: Aplastic Anemia, Myelodysplastic Syndromes and PNH. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2005. 19 p.
This booklet provides information about coping with bone marrow diseases, such as aplastic anemia or myelodysplastic syndromes (MDS), for patients and their families. Topics include reactions to illness, reactions from others, changes to expect within the family, learning to cope, protecting relationships, the hospital setting, coping with hospital life, policies and procedures, changes in the patient’s appearance and behavior, the health care team, nights and weekends, tips for frequent hospital stays, managing family life, other patients and their families, discharge from the hospital, helping children cope, special challenges for single parent families, and special challenges for blended families. Throughout the book, the authors stress the importance of communication. Adjustments are easier for everyone if the patient, other family members, and the hospital personnel can talk openly about the patient’s treatment and the accompanying life changes that are required. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research. 5 references.
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Iron Overload in Patients with Bone Marrow Disease. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2005. 2 p.
This fact sheet discusses the problem of iron overload in patients with bone marrow disease. Those who develop iron overload from transfusions are dependent on red blood cell transfusions and most need ongoing transfusions due to lack of adequate blood production. Iron can be removed from the body by drugs called chelators, which have the ability to bind with the iron. The drug approved in the United States for this purpose is called deferoxamine, which is given intravenously or subcutaneously. The author reviews the diagnostic tests that monitor the body’s iron stores, discusses the administration and dosage of deferoxamine, and briefly mentions the development of new oral agents under evaluation for use in patients with transfusional iron overload. Readers are encouraged to contact the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research, for more information.
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Managing Treatment Decisions for Bone Marrow Failure Disease: Aplastic Anemia, Myelodysplastic Syndromes, Paroxysmal Nocturnal Hemoglobinuria. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2005. 11 p.
Aplastic anemia is a rare, non-contagious and often life-threatening disorder that results from the unexplained failure of the bone marrow to produce blood cells. MDS is a bone marrow failure disorder that is similar to aplastic anemia. This booklet provides information for people recently diagnosed with bone marrow diseases, such as aplastic anemia, paroxysmal nocturnal hemoglobinuria (PNH), or myelodysplastic syndromes (MDS), and helps them make important treatment decisions. Topics include managing the disease, finding an expert, the importance of communicating successfully with one’s health care provider (including discussing any conflicts with the medical team), informed consent, considering participation in clinical trials, supplements (such as vitamins or special diets) from sources other than the primary health care provider, the importance of recordkeeping, the role of patient education, and talking to other patients. The booklet includes a list of 16 suggested questions to ask the health care provider about any recommended treatment. Blank space is also provided for patients to record any other questions they may want to remember. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research.
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Myelodysplastic Syndromes: Basic Explanations. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2005. 23 p.
Myelodysplastic syndromes (MDS) occur when the bone marrow stem cells malfunction. This results in the production of too many defective blood cells and not enough normal blood cells. This booklet provides general information about MDS for patients and their families. Topics include how MDS occurs, the symptoms and diagnosis, how it is treated, what to expect after a diagnosis of MDS, how the body’s blood system works (both normally and in the presence of MDS), how the body produces blood cells and fights infection, the different types of MDS, the similarities and differences between MDS and aplastic anemia, the causes and risk factors of MDS, treatment options (including supportive care, growth factors, immunosuppressive drug therapy, chemotherapy, and bone marrow transplants), blood transfusions, clinical research studies and emotional issues. The section on blood transfusions covers red blood cell transfusions, the importance of irradiated and leukocyte-reduced blood, platelet transfusions, white blood cell transfusions, transfusion reactions, the risks of transfusion contamination, and the use of topical anesthetic during transfusions. A glossary of terms is provided, with terms that are included in the glossary also highlighted in the text. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research. 1 figure. 3 tables.
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PNH: Basic Explanations. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2004. 11 p.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare but potentially serious blood disease affecting people of all ages. In PNH, a mutation in the genetic material of a stem cell affects a specific gene (PIG-A); all red cells, white cells, and platelets produced by the diseased stem cell carry the defect that causes symptoms associated with this disease. This booklet provides general information about PNH for patients and their families. Topics include how PNH occurs, the symptoms and diagnosis, how it is treated, what to expect after a diagnosis of PNH, how the body’s blood system works (both normally and in the presence of PNH), how the body produces blood cells and fights infection, the similarities and differences between PNH and aplastic anemia, the causes and risk factors of PNH, treatment options (including conservative treatment and bone marrow transplantation), prognosis, blood transfusions, and emotional issues. The section on blood transfusions covers red blood cell transfusions, the importance of irradiated and leukocyte-reduced blood, platelet transfusions, white blood cell transfusions, transfusion reactions, the risks of transfusion contamination, and the use of topical anesthetic during transfusions. A glossary of terms is provided, with terms that are included in the glossary also highlighted in the text. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research. 2 tables.
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Talking to a Child with Bone Marrow Disease. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2004. 29 p.
This booklet was created to be read aloud by a parent, caregiver, or health care professional to a child patient of any age. It was written to encourage children with bone marrow diseases to ask questions and express their feelings. Each page of text and illustration are designed to identify different topics, including emotions, the importance of talking with others about one’s feelings, different ways to express feelings, the role of the bone marrow in making blood cells, hospital care and the people who work there, taking medicine, family dynamics, recreational activities, talking with friends and classmates about the illness, and coping with being ill. Each page includes questions designed to draw the child into conversation, to illuminate hidden fears and misconceptions that can be clarified. The booklet is illustrated with full-color line drawings of children in a variety of health care and home settings. The booklet concludes with a few pages of blank lines for readers to write out their thoughts. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research.
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Teachers and School Nurses Guide to Bone Marrow Failure Disease. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2004. 5 p.
Aplastic anemia is a rare, non-contagious and often life-threatening disorder that results from the unexplained failure of the bone marrow to produce blood cells. MDS is a bone marrow failure disorder that is similar to aplastic anemia. This booklet provides information about students coping with bone marrow diseases, such as aplastic anemia, paroxysmal nocturnal hemoglobinuria (PNH), or myelodysplastic syndromes (MDS), for teachers and school nurses who may be working with these patients. The brochure is designed to help educators understand their role in helping students, their families, and the student’s classmates to cope with bone marrow failure diseases. Topics include a brief definition of aplastic anemia and MDS, the common symptoms of these conditions, how to support the child’s emotional needs, answering common questions that classmates may ask, ideas for classroom discussion, classroom activities that may help children understand the child’s illness, dealing with a child in school who is terminally ill, the role of the school nurse, and working with the student’s parents. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research.
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Acquired Aplastic Anemia: Basic Explanations. Annapolis, MD: Aplastic Anemia and MDS International Foundation, Inc. 2003. 14 p.
Aplastic anemia is a condition that occurs when the bone marrow stops making enough blood cells, including red blood cells, white blood cells, and platelets. This booklet provides general information about aplastic anemia for patients and their families. Topics include how aplastic anemia occurs, the symptoms and diagnosis, how it is treated, what to expect after a diagnosis of aplastic anemia, how the body’s blood system works (both normally and in the presence of aplastic anemia), how the body produces blood cells and fights infection, the different types of aplastic anemia, the similarities and differences between aplastic anemia and myelodysplastic syndromes, the causes and risk factors of aplastic anemia, treatment options (including bone marrow transplantation, immunosuppressive drug therapy, and growth factors), the use of transfusions to treat aplastic anemia, and emotional issues. The section on blood transfusions covers red blood cell transfusions, the importance of irradiated and leukocyte-reduced blood, platelet transfusions, white blood cell transfusions, transfusion reactions, the risks of transfusion contamination, and the use of topical anesthetic during transfusions. A glossary of terms is provided, with terms that are included in the glossary also highlighted in the text. The back cover of the booklet describes the goals and activities of the Aplastic Anemia and MDS International Foundation, an organization that fights bone marrow diseases through patient support and research. 1 table.
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