Journal article
Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib
CY Cheah, K Burbury, JF Apperley, F Huguet, V Pitini, M Gardembas, DM Ross, D Forrest, P Genet, P Rousselot, N Patton, G Smith, CE Dunbar, S Ito, RCT Aguiar, O Odenike, A Gimelfarb, NCP Cross, JF Seymour
Blood | Published : 2014
Abstract
Myeloid neoplasms and eosinophilia with rearrangements of PDGFRB are uncommon Philadelphia-negative myeloproliferative neoplasms. Patients are typically male, with morphologic features of a Philadelphia-negative chronic myeloproliferative syndrome or chronicmyelomonocytic leukemia with eosinophilia. Reciprocal translocations involving PDGFRB result in fusion genes with constitutively activated receptor tyrosine kinase sensitive to inhibition with imatinib. We present an updated and expanded analysis of a cohort of 26 such patients treated with imatinib. After a median follow-up of 10.2 years (range, 1.8-17 years), the 10-year overall survival rate was 90% (95% confidence interval, 64%-97%); ..
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Awarded by National Heart, Lung, and Blood Institute
Funding Acknowledgements
The authors thank Dr N. Carvalho and Dr E. Velloso (Sao Paulo, Brazil) for clinical updates and Professor Francois Xavier Mahon for providing molecular analysis for French patients. This work was funded in part by the Victorian Cancer Agency (grant CTCB11_18) and the Haematology Society of Australia and New Zealand (New Investigator Scholarship).