دورية أكاديمية
The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management
| العنوان: | The FIP1L1-PDGFRalpha fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management |
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| المؤلفون: | Gotlib, Jason, Cools, Jan, Malone, James M, Schrier, Stanley L, Gilliland, D Gary, Coutré, Steven E |
| بيانات النشر: | W.B. Saunders |
| سنة النشر: | 2004 |
| المجموعة: | KU Leuven: Lirias |
| مصطلحات موضوعية: | Algorithms, Antineoplastic Agents, Cytogenetics, Humans, Hypereosinophilic Syndrome, Piperazines, Prognosis, Protein-Tyrosine Kinase, Pyrimidines, Receptor, Platelet-Derived Growth Factor alpha, Research Support, Non-U.S. Gov't, U.S. Gov't, P.H.S, T-Lymphocytes, mRNA Cleavage and Polyadenylation Factors |
| الوصف: | Idiopathic hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL) comprise a spectrum of indolent to aggressive diseases characterized by unexplained, persistent hypereosinophilia. These disorders have eluded a unique molecular explanation, and therapy has primarily been oriented toward palliation of symptoms related to organ involvement. Recent reports indicate that HES and CEL are imatinib-responsive malignancies, with rapid and complete hematologic remissions observed at lower doses than used in chronic myelogenous leukemia (CML). These BCR-ABL-negative cases lack activating mutations or abnormal fusions involving other known target genes of imatinib, implicating a novel tyrosine kinase in their pathogenesis. A bedside-to-benchtop translational research effort led to the identification of a constitutively activated fusion tyrosine kinase on chromosome 4q12, derived from an interstitial deletion, that fuses the platelet-derived growth factor receptor-alpha gene (PDGFRA) to an uncharacterized human gene FIP1-like-1 (FIP1L1). However, not all HES and CEL patients respond to imatinib, suggesting disease heterogeneity. Furthermore, approximately 40% of responding patients lack the FIP1L1-PDGFRA fusion, suggesting genetic heterogeneity. This review examines the current state of knowledge of HES and CEL and the implications of the FIP1L1-PDGFRA discovery on their diagnosis, classification, and management. ; status: published |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| تدمد: | 0006-4971 |
| العلاقة: | Blood vol:103 issue:8 pages:2879-2891; https://lirias.kuleuven.be/handle/123456789/7929Test; http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=15070659Test |
| الإتاحة: | https://lirias.kuleuven.be/handle/123456789/7929Test http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=15070659Test |
| رقم الانضمام: | edsbas.111D0947 |
| قاعدة البيانات: | BASE |
Full Text Finder | تدمد: | 00064971 |
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