Imatinib Therapy in Clonal Eosinophilic Disorders, Including Systemic Mastocytosis

Article

DOI: 10.1532/IJH97.04046

Cite this article as:
Tefferi, A. & Pardanani, A. Int J Hematol (2004) 79: 441. doi:10.1532/IJH97.04046

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 (≥1500/μL 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. Thebone marrow histologic phenotype of these imatinib-sensitive eosinophilic disorders includes systemic mastocytosis, chronic eosinophilic leukemia, chronic myelomonocytic leukemia, and atypical chronic myeloproliferative disorder.

Key words

Eosinophilia Hypereosinophilic disorder Mastocytosis FIP1L1-PDGFRA 

Copyright information

© The Japanese Society of Hematology 2004

Authors and Affiliations

  1. 1.Division of Hematology and Internal MedicineMayo ClinicRochesterUSA

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