Imatinib mesylate can induce complete molecular remission in FIP1L1-PDGFR-a positive idiopathic hypereosinophilic syndrome.
نویسندگان
چکیده
236 the 5’-flanking region of the IL-6 and IL-8 genes do not act independently of one another but as part of an extended promoter haplotype. In summary, our data demonstrate that the assessment of IL-6 and IL-8 levels in febrile neutropenic children is of limited diagnostic value and is not improved by genotyping for promoter polymorphisms. Thomas Lehrnbecher,* Gudrun Fleischhack,° Mitra Hanisch,* Frank Deinlein, Arne Simon,° Toralf Bernig, Stephen J. Chanock, Thomas Klingebiel* Departments of Pediatric Hematology and Oncology, University of Frankfurt,* Bonn,° Würzburg; Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA Funding: this study was supported by the Wilhelm Sander-Stiftung. Acknowledgments: we thank the medical and nursing staff of the Departments of Pediatric Hematology and Oncology, Universities of Bonn, Frankfurt and Würzburg, Germany.
منابع مشابه
FIP1L1/PDGFR -Associated Systemic Mastocytosis
Since the identification of the FIP1L1/PDGFRA fusion gene as a pathogenic cause of the hypereosinophilic syndrome (HES), the importance of the molecular classification of HES leading to the diagnosis of chronic eosinophilic leukemia (CEL) has been recognized. As a result, a new category, ‘myeloid and lymphoid neoplasm with eosinophilia and abnormalities in PDGFRA, PDGFRB or FGFR1’, has recently...
متن کاملThe efficacy of imatinib mesylate in patients with FIP1L1-PDGFRalpha-positive hypereosinophilic syndrome. Results of a multicenter prospective study.
BACKGROUND AND OBJECTIVES The hypereosinophilic syndrome (HES) may be associated with the fusion of the platelet derived growth factor receptor a (PDGFRalpha) gene with the FIP1L1 gene in chromosome 4 coding for a constitutively activated PDGFRalpha tyrosine kinase. These cases with FIP1L1-PDGFRalpha rearrangement have been reported to be very sensitive to the tyrosine kinase inhibitor imatinib...
متن کاملTowards a rational treatment of essential thrombocythemia, despite limited evidence and old prejudices.
137 interleukin-5-producing T cells in idiopathic eosinophilia. New Engl J Med 1999;341:1112-20. 7. Roufosse F, Schandene L, Cogan E. Idiopathic eosinophilia. N Engl J Med 1999;342:660. 8. Bassan R, Locatelli G, Borleri G, Salvi A, Barbui T. Immunophenotypic evaluation of circulating T-cell clones in hypereosinophilic syndromes with or without abnormal CD3 and CD4 lymphocytes. Haematologica 200...
متن کاملIdiopathic hypereosinophilic syndrome presenting with severe vasculitis successfully treated with imatinib
Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment wi...
متن کاملCHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy.
Imatinib mesylate is effective in the treatment of hematologic malignancies that are characterized by either abl- or PDGFR beta- activating mutations. The drug is also active in a subset of patients with eosinophilic disorders and systemic mast cell disease (SMCD). Recently, a novel tyrosine kinase that is generated from fusion of the Fip1-like 1 (FIP1L1) and PDGFR alpha (PDGFRA) genes has been...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Haematologica
دوره 89 2 شماره
صفحات -
تاریخ انتشار 2004