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

Imatinib in myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB in chronic or blast phase
Imatinib in myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRB in chronic or blast phase
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.
0939-5555
1463-1470
Jawhar, Mohamad
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Naumann, Nicole
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Schwaab, Juliana
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Baurmann, Herrad
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Casper, Jochen
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Dang, Tu-anh
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Dietze, Lutz
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Döhner, Konstanze
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Hänel, Annette
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Lathan, Bernd
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Link, Hartmut
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Lotfi, Sina
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Maywald, Ole
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Mielke, Stephan
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Müller, Lothar
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Platzbecker, Uwe
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Prümmer, Otto
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Thomssen, Henrike
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Töpelt, Karin
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Panse, Jens
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Vieler, Tom
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Hofmann, Wolf-Karsten
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Haferlach, Torsten
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Haferlach, Claudia
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Fabarius, Alice
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Hochhaus, Andreas
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Cross, Nicholas C.P.
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Reiter, Andreas
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Metzgeroth, Georgia
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Jawhar, Mohamad
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Naumann, Nicole
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Schwaab, Juliana
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Baurmann, Herrad
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Casper, Jochen
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Dang, Tu-anh
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Dietze, Lutz
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Döhner, Konstanze
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Hänel, Annette
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Lathan, Bernd
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Link, Hartmut
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Lotfi, Sina
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Maywald, Ole
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Mielke, Stephan
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Müller, Lothar
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Platzbecker, Uwe
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Prümmer, Otto
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Thomssen, Henrike
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Töpelt, Karin
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Panse, Jens
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Vieler, Tom
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Hofmann, Wolf-Karsten
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Haferlach, Torsten
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Haferlach, Claudia
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Fabarius, Alice
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Hochhaus, Andreas
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Cross, Nicholas C.P.
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Reiter, Andreas
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Metzgeroth, Georgia
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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).

Record type: Article

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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PDGFRB_Manuscript_Jawhar_final (002) - Accepted Manuscript
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Accepted/In Press date: 7 July 2017
e-pub ahead of print date: 19 July 2017
Published date: September 2017

Identifiers

Local EPrints ID: 412792
URI: http://eprints.soton.ac.uk/id/eprint/412792
ISSN: 0939-5555
PURE UUID: 1d0628af-0a95-4d8f-a15c-d14a27831523
ORCID for Nicholas C.P. Cross: ORCID iD orcid.org/0000-0001-5481-2555

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Date deposited: 01 Aug 2017 16:31
Last modified: 16 Mar 2024 05:34

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Contributors

Author: Mohamad Jawhar
Author: Nicole Naumann
Author: Juliana Schwaab
Author: Herrad Baurmann
Author: Jochen Casper
Author: Tu-anh Dang
Author: Lutz Dietze
Author: Konstanze Döhner
Author: Annette Hänel
Author: Bernd Lathan
Author: Hartmut Link
Author: Sina Lotfi
Author: Ole Maywald
Author: Stephan Mielke
Author: Lothar Müller
Author: Uwe Platzbecker
Author: Otto Prümmer
Author: Henrike Thomssen
Author: Karin Töpelt
Author: Jens Panse
Author: Tom Vieler
Author: Wolf-Karsten Hofmann
Author: Torsten Haferlach
Author: Claudia Haferlach
Author: Alice Fabarius
Author: Andreas Hochhaus
Author: Andreas Reiter
Author: Georgia Metzgeroth

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