Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib

Chan Y. Cheah, Kate Burbury, Jane F. Apperley, Francoise Huguet, Vincenzo Pitini, Martine Gardembas, David M. Ross, Donna Forrest, Philippe Genet, Philippe Rousselot, Nigel Patton, Graeme Smith, Cynthia E. Dunbar, Sawa Ito, Ricardo C.T. Aguiar, Olatoyosi Odenike, Alla Gimelfarb, Nicholas C.P. Cross, John F. Seymour

Research output: Contribution to journalArticlepeer-review

73 Scopus citations

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%); after median imatinib duration of 6.6 years (range, 0.1-12 years), the 6-year progression-free survival rate was 88% (95% confidence interval, 65%-96%). Of the patients, 96% responded; no patients who achieved a complete cytogenetic (n = 13) or molecular (n = 8) remission lost their response or progressed to blast crisis. Imatinib is well-tolerated and achieves excellent long-term responses in patients with PDGFRB rearrangements.

Original languageEnglish (US)
Pages (from-to)3574-3577
Number of pages4
JournalBlood
Volume123
Issue number23
DOIs
StatePublished - Jun 5 2014

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

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