TY - JOUR
T1 - Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long-term remissions with imatinib
AU - Cheah, Chan Y.
AU - Burbury, Kate
AU - Apperley, Jane F.
AU - Huguet, Francoise
AU - Pitini, Vincenzo
AU - Gardembas, Martine
AU - Ross, David M.
AU - Forrest, Donna
AU - Genet, Philippe
AU - Rousselot, Philippe
AU - Patton, Nigel
AU - Smith, Graeme
AU - Dunbar, Cynthia E.
AU - Ito, Sawa
AU - Aguiar, Ricardo C.T.
AU - Odenike, Olatoyosi
AU - Gimelfarb, Alla
AU - Cross, Nicholas C.P.
AU - Seymour, John F.
N1 - Copyright:
Copyright 2014 Elsevier B.V., All rights reserved.
PY - 2014/6/5
Y1 - 2014/6/5
N2 - 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.
AB - 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.
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U2 - 10.1182/blood-2014-02-555607
DO - 10.1182/blood-2014-02-555607
M3 - Article
C2 - 24687085
AN - SCOPUS:84902168976
VL - 123
SP - 3574
EP - 3577
JO - Blood
JF - Blood
SN - 0006-4971
IS - 23
ER -