Protocol Number: 04-I-0286

Title:

A Longitudinal Study of Familial Hyperosinophilia (FE): Natural History and Markers of Disease Progression

Number:

04-I-0286

Summary:

Affected members of a previously studied familial hypereosinophilia (FE) will be admitted on this protocol. A thorough clinical evaluation will be performed yearly with emphasis on potential sequelae of eosinophil-mediated tissue damage. Blood cells and/or serum will also be collected to provide reagents (such as DNA, RNA, and specific antibodies) for use in the laboratory to address issues related to the genetic and immunologic basis of FE as well as its pathogenesis. It is anticipated that patients will undergo a more extensive evaluation than is generally available and that the specimens collected from them will prove to be valuable reagents for laboratory studies related to eosinophilia, eosinophil activation and function. While the study is not designed to address the question of therapy for FE, in patients for whom medical therapy is indicated (for either the hypereosinophilia itself or its sequelae), appropriate treatment will be instituted by our clinical service or the patients' local physicians. No experimental chemotherapy is involved in this protocol.

Sponsoring Institute:

National Institute of Allergy and Infectious Diseases (NIAID)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

-genetically related member of the previously identified family with FE

-documented eosinophilia greater than 1,500/mm(3) on at least two occasions

EXCLUSION CRITERIA:

-an alternative explanation for eosinophilia greater than 1,500/mm(3) (ex. Hypersensitivity reaction, parasitic infection)

Special Instructions: Currently Not Provided

Keywords:

Genetic

Eosinophils

Hypereosinophilia

Recruitment Keywords:

None

Conditions:

Eosinophilia

Investigational Drug(s):

None

Investigational Device(s):

None

Contacts:

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

Citations:

Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994 May 15;83(10):2759-79. Review. No abstract available.

Cools J, DeAngelo DJ, Gotlib J, Stover EH, Legare RD, Cortes J, Kutok J, Clark J, Galinsky I, Griffin JD, Cross NC, Tefferi A, Malone J, Alam R, Schrier SL, Schmid J, Rose M, Vandenberghe P, Verhoef G, Boogaerts M, Wlodarska I, Kantarjian H, Marynen P, Coutre SE, Stone R, Gilliland DG. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome.

N Engl J Med. 2003 Mar 27;348(13):1201-14.

Klion AD, Noel P, Akin C, Law MA, Gilliland DG, Cools J, Metcalfe DD, Nutman TB. Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness. Blood. 2003 Jun 15;101(12):4660-6. Epub 2003 Apr 03.

• Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
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Warren Grant Magnuson Clinical Center (CC)
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Bethesda, Maryland 20892. Last update: 10/27/2004