PMID: 15239393Jul 9, 2004Paper

Imatinib therapy in clonal eosinophilic disorders, including systemic mastocytosis

International Journal of Hematology
A Tefferi, A Pardanani

Abstract

Primary (nonreactive) eosinophilia is operationally classified as either a "clonal" or an "idiopathic" process. Clonal eosinophilia stipulates the presence of cytogenetic, molecular, or bone marrow histologic evidence of acute leukemia or a chronic myeloid disorder. Idiopathic eosinophilia is a diagnosis of exclusion that is made after ruling out both "secondary" (reactive) and clonal eosinophilia. Hypereosinophilic syndrome is a subclass of idiopathic eosinophilia that requires the documentation of both sustained eosinophilia (> or = 1500/microL for at least 6 months) and target-organ damage. A series of novel observations in the last 5 years have warranted a refined approach to the diagnosis as well as the treatment of clonal eosinophilic disorders, including systemic mastocytosis. At the center of these new developments are mutations involving the platelet-derived growth factor receptor genes (PDGFRA and PDGFRB), which have been pathogenetically linked to clonal eosinophilia, and their presence predicts complete as well as durable treatment responses to imatinib mesylate. The bone marrow histologic phenotype of these imatinib-sensitive eosinophilic disorders includes systemic mastocytosis, chronic eosinophilic leukemia, chro...Continue Reading

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Citations

Aug 26, 2006·Immunology and Allergy Clinics of North America·Peter Valent
Jun 20, 2006·Best Practice & Research. Clinical Haematology·A PardananiP Valent
Aug 23, 2005·Leukemia Research·Hermine AgisHans-Peter Horny
Apr 23, 2011·Blood·Mahadeo A SukhaiAaron D Schimmer
Dec 29, 2004·Leukemia & Lymphoma·Peter ValentDean D Metcalfe
Jan 11, 2020·Journal of the National Cancer Institute·Keith T FlahertyUNKNOWN NCI-MATCH Team

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