Imatinib in myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB in chronic or blast phase - PubMed (original) (raw)

Clinical Trial

. 2017 Sep;96(9):1463-1470.

doi: 10.1007/s00277-017-3067-x. Epub 2017 Jul 19.

Nicole Naumann 1 2, Juliana Schwaab 1 2, Herrad Baurmann 3, Jochen Casper 4, Tu-Anh Dang 5, Lutz Dietze 6, Konstanze Döhner 7, Annette Hänel 8, Bernd Lathan 9, Hartmut Link 10, Sina Lotfi 11, Ole Maywald 12, Stephan Mielke 13, Lothar Müller 14, Uwe Platzbecker 15, Otto Prümmer 16, Henrike Thomssen 17, Karin Töpelt 18, Jens Panse 19, Tom Vieler 20, Wolf-Karsten Hofmann 1 2, Torsten Haferlach 21, Claudia Haferlach 21, Alice Fabarius 1 2, Andreas Hochhaus 22, Nicholas C P Cross 23 24, Andreas Reiter 25 26, Georgia Metzgeroth 1 2

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Clinical Trial

Imatinib in myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB in chronic or blast phase

Mohamad Jawhar et al. Ann Hematol. 2017 Sep.

Abstract

We evaluated clinical characteristics and outcome on imatinib of 22 patients with myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB. Median age was 49 years (range 20-80), 91% were male. Fifteen different PDGFRB fusion genes were identified. Eosinophilia was absent in 4/19 (21%) cases and only 11/19 (58%) cases had eosinophils ≥1.5×109/L. On imatinib, 17/17 (100%) patients in chronic phase achieved complete hematologic remission after median 2 months (range 0-13)​. Complete cytogenetic remission and/or complete molecular remission by RT-PCR were achieved in 12/13 (92%) and 12/14 patients (86%) after median 10 (range 3-34) and 19 months (range 7-110), respectively. In patients with blast phase (myeloid, n = 2; lymphoid, n = 3), treatment included combinations of imatinib (n = 5), intensive chemotherapy (n = 3), and/or allogeneic stem cell transplantation (n = 3). All 3 transplanted patients (complex karyotype, n = 2) experienced early relapse. Initially, patients were treated with imatinib 400 mg/day (n = 15) or 100 mg/day (n = 7), the dose was reduced from 400 mg/day to 100 mg/day during follow-up in 9 patients. After a median treatment of 71 months (range 1-135), the 5-year survival rate was 83%; 4/22 (18%) patients died (chronic phase; n = 2; blast phase, n = 2) due to progression (n = 3) or comorbidity while in remission (n = 1). Of note, 3/4 patients had a complex karyotype. In summary, the most important characteristics of myeloid/lymphoid neoplasms with rearrangement of PDGFRB include (a) male predominance, (b) frequent lack of hypereosinophilia,

Keywords: Clonal eosinophilia; Fusion gene; Imatinib; MPN; PDGFRB rearrangement.

(c) presentation in chronic or blast phase, (d) rapid responses and long-term remission on low-dose imatinib, and (e) possible adverse prognostic impact of a complex karyotype.

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