Jawhar, Mohamad, Naumann, Nicole, Schwaab, Juliana, Baurmann, Herrad, Casper, Jochen, Dietze, Lutz, Doehner, Konstanze, Haenel, Annette, Lathan, Bernd, Link, Hartmut, Lotfi, Sina, Maywald, Ole, Mielke, Stephan ORCID: 0000-0002-8325-9215, Mueller, Lothar, Platzbecker, Uwe, Pruemmer, Otto, Thomssen, Henrike, Toepelt, 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. Ann. Hematol., 96 (9). S. 1463 - 1471. NEW YORK: SPRINGER. ISSN 1432-0584

Full text not available from this repository.

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 >= 1.5x10(9)/L. On imatinib, 17/17 (100%) patients in chronic phase achieved complete hematologic remission after median 2 months (range 0-13)aEuroi. 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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Jawhar, MohamadUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Naumann, NicoleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schwaab, JulianaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Baurmann, HerradUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Casper, JochenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dietze, LutzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Doehner, KonstanzeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haenel, AnnetteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lathan, BerndUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Link, HartmutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lotfi, SinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Maywald, OleUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mielke, StephanUNSPECIFIEDorcid.org/0000-0002-8325-9215UNSPECIFIED
Mueller, LotharUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Platzbecker, UweUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pruemmer, OttoUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Thomssen, HenrikeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Toepelt, KarinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Panse, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vieler, TomUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hofmann, Wolf-KarstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haferlach, TorstenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haferlach, ClaudiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fabarius, AliceUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hochhaus, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Cross, Nicholas C. P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reiter, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Metzgeroth, GeorgiaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-221214
DOI: 10.1007/s00277-017-3067-x
Journal or Publication Title: Ann. Hematol.
Volume: 96
Number: 9
Page Range: S. 1463 - 1471
Date: 2017
Publisher: SPRINGER
Place of Publication: NEW YORK
ISSN: 1432-0584
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
TERM-FOLLOW-UP; MYELOID NEOPLASMS; FUSION GENES; LEUKEMIA; DISCONTINUATION; RESISTANCE; REMISSION; MESYLATEMultiple languages
HematologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/22121

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item