Journal article

Durable responses to imatinib in patients with PDGFRB fusion gene-positive and BCR-ABL-negative chronic myeloproliferative disorders

Marianna David, Nicholas CP Cross, Sonja Burgstaller, Andrew Chase, Claire Curtis, Raymond Dang, Martine Gardembas, John M Goldman, Francis Grand, George Hughes, Francoise Huguet, Louise Lavender, Grant A McArthur, Francois X Mahon, Giorgio Massimini, Junia Melo, Philippe Rousselot, Robin J Russell-Jones, John F Seymour, Graeme Smith Show all

BLOOD | AMER SOC HEMATOLOGY | Published : 2007

Abstract

Fusion genes derived from the platelet-derived growth factor receptor beta (PDGFRB) or alpha (PDGFRA) play an important role in the pathogenesis of BCR-ABL-negative chronic myeloproliferative disorders (CMPDs). These fusion genes encode constitutively activated receptor tyrosine kinases that can be inhibited by imatinib. Twelve patients with BCR-ABL-negative CMPDs and reciprocal translocations involving PDGFRB received imatinib for a median of 47 months (range, 0.1-60 months). Eleven had prompt responses with normalization of peripheral-blood cell counts and disappearance of eosinophilia; 10 had complete resolution of cytogenetic abnormalities and decrease or disappearance of fusion transcri..

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