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 and Metzgeroth, Georgia (2017) Imatinib in myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB in chronic or blast phase. Annals of Hematology, 96 (9), 1463-1470. (doi:10.1007/s00277-017-3067-x).
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.
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