Protocol Number: 06-I-0032

Title:

Pilot Study of Omalizumab (Xolair) in Hyper IgE (Job's) Syndrome

Number:

06-I-0032

Summary:

This study will evaluate the safety and effectiveness of a laboratory-made antibody called omalizumab in patients with Job's syndrome, or hyper-IgE syndrome (HIES). Patients with HIES have very high levels of IgE antibody, a protein made by white blood cells. IgE plays an important role in starting allergic reactions in the body and may be related to some HIES symptoms, such as skin rashes and asthma. Patients also have frequent lung infections, easily broken bones and other symptoms. Omalizumab, which is approved to treat allergic asthma, is directed against IgE. This study will see if blocking IgE with omalizumab in HIES patients is safe and if it can reduce patients' IgE count. It will also look at how the body handles omalizumab and how it affects patients' symptoms.

Patients 6 years of age or older with HIES may be eligible for this study. Each candidate is screened with a medical history, physical examination, skin examination and blood test.

Participants receive an injection of omalizumab under the skin once every 2 weeks for 6 doses. At the time of each injection, patients are examined by a doctor, answer questions about their symptoms and have a blood sample drawn. After the sixth dose, patients have a physical examination, blood tests, skin examination and lung function tests. At follow-up visits scheduled 2, 4 and 6 months after the last dose of omalizumab, patients have a physical examination, answer questions about their symptoms, and have a blood sample drawn. Patients who show a significant response to omalizumab stay off the drug for 3 months after the last dose and then discuss with their study doctor and referring doctor about continuing the medicine.

Sponsoring Institute:

National Institute of Allergy and Infectious Diseases (NIAID)

Recruitment Detail

Type: Participants currently recruited/enrolled

Gender: Male & Female

Referral Letter Required: Yes

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

1. All Subjects must be at least 6 years of age and no older than 76 years of age (as per original safety studies).

2. All subjects must meet the established diagnostic criteria for HIES as determined by the NIH scoring system, with a score greater than 40.

3. Baseline values within the following laboratory ranges:

White blood cell count greater than or equal to 3,300 cells/microliter.

Absolute neutrophil count greater than or equal to 1,000 cells/microliter.

Hemoglobin greater than or equal to 10 g/dL.

Platelet count greater than or equal to 100,000 platelets/micoliter.

4. Women of childbearing potential only: negative serum beta-hCG. Both men and women and their partners must agree to practice abstinence or effective contraception from initiation of the protocol and for 2 months following the last dose of the study drug (effective contraception methods include abstinence, surgical sterilization of either partner, barrier methods such as diaphragm, condom, cap, or sponge, or hormonal contraception).

5. Weight (Kg) x serum IgE (IU/mL) less than or equal to 63,000 (per dosing restrictions) for initial safety study, then, as above less than 2,000,000, greater than 63,000, then as above greater than 63,000, less than 125,000.

6. Patients in tier IV must be 16 years or older.

7. Preference will be given to patients with dermatitis.

8. Subjects (guardians for younger patients) must be able to give informed consent (or assent as appropriate).

9. Subjects must already be signed to other NIH studies on HIES.

10. Subjects must be willing to maintain their current regimens for skin care, prophylactic antibiotics (if applicable), and any asthma related oral or inhaled medications. Albuterol rescue medications may be used as needed.

11. Patients participating in protocols at the National Institutes of Health are expected to have a primary physician outside of the NIH.

EXCLUSION CRITERIA:

1. Pregnant or nursing women.

2. HIV positive diagnosis.

3. Use of any other investigational agent within 30 days of the study.

4. Any condition that, in the investigator's opinion, places the patient at undue risk by participating in the study.

5. Previous anaphylaxis to the study medication.

Special Instructions:

Currently Not Provided

Keywords:

Phenotype

Monoclonal

Immunoglobulin

Immunodeficiency

Dermatitis

Recruitment Keyword(s):

Job's Syndrome

Hyper-IgE Syndrome

HIES

Condition(s):

Hyper-IgE syndrome

Job's Syndrome

Investigational Drug(s):

Omalizumab (Xolair)

Investigational Device(s):

None

Intervention(s):

Drug: Omalizumab (Xolair)

Supporting Site:

National Institute of Allergy and Infectious Diseases

Contact(s):

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citation(s):

Lee M, Hodges WG, Huggins TL, Lee EL. Eosinophilic gastroenteritis. South Med J. 1996 Feb;89(2):189-94.

Kelly KJ. Eosinophilic gastroenteritis. J Pediatr Gastroenterol Nutr. 2000;30 Suppl:S28-35. Review.

Park HS, Kim HS, Jang HJ. Eosinophilic gastroenteritis associated with food allergy and bronchial asthma. J Korean Med Sci. 1995 Jun;10(3):216-9.

• Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
• Technical questions regarding the Clinical Center web site, please contact the Department of Networks and Applications, CC.

Search The Studies | Help | Questions |
Clinical Center Home | NIH Home

National Institutes of Health Clinical Center Bethesda, Maryland 20892. Last update: 09/20/2008