학술논문
Imatinib in myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB in chronic or blast phase
Document Type
Original Paper
Author
Jawhar, Mohamad; Naumann, Nicole; Schwaab, Juliana; Baurmann, Herrad; Casper, Jochen; Dang, Tu-Anh; Dietze, Lutz; Döhner, Konstanze; Hänel, Annette; Lathan, Bernd; Link, Hartmut; Lotfi, Sina; Maywald, Ole; Mielke, Stephan; Müller, Lothar; Platzbecker, Uwe; Prümmer, Otto; Thomssen, Henrike; Töpelt, Karin; Panse, Jens; Vieler, Tom; Hofmann, Wolf-Karsten; Haferlach, Torsten; Haferlach, Claudia; Fabarius, Alice; Hochhaus, Andreas; Cross, Nicholas C.P.; Reiter, Andreas; Metzgeroth, Georgia
Source
Annals of Hematology. September 2017 96(9):1463-1470
Subject
Language
English
ISSN
0939-5555
1432-0584
1432-0584
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, (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.