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Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long term remissions with imatinib

Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long term remissions with imatinib
Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long term remissions with imatinib
Myeloid neoplasms and eosinophilia with rearrangements of PDGFRB are uncommon Philadelphia negative myeloproliferative neoplasms. Patients are typically male, with morphologic features of a Ph- chronic myeloproliferative syndrome or chronic myelomonocytic leukemia with eosinophilia. Reciprocal translocations involving PDGFRB result in fusion genes with constitutively activated receptor tyrosine kinase sensitive to inhibition with imatinib. We present an updated and expanded analysis of a cohort of 26 such patients treated with imatinib. After a median follow-up of 10.2 (range 1.8 - 17) years, the 10-year overall survival rate was 90% (95%CI 64-97%); after median imatinib duration of 6.6 (range 0.1 - 12) years the 6-year progression-free survival rate was 88% (95% CI; 65-96%). 96% patients responded; no patients who achieved a complete cytogenetic (n=13) or molecular (n=8) remission lost their response or progressed to blast crisis. Imatinib is well tolerated and achieves excellent long-term responses in patients with PDGFRB rearrangements.
0006-4971
3574-3577
Cheah, C.Y.
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Burbury, K.
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Apperley, J.F.
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Huguet, F.
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Pitini, V.
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Gardembas, M.
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Ross, D.M.
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Forrest, D.
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Genet, P.
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Rousselot, P.
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Patton, N.
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Smith, G.
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Dunbar, C.E.
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Ito, S.
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Aguiar, R.C.T.
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Odenike, O.
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Gimelfarb, A.
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Cross, N.C.P.
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Seymour, J.F.
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Cheah, C.Y.
a8acd70f-08fc-46d6-8be9-f7205f451094
Burbury, K.
ffcdcf65-edb6-4796-b3f1-6d4f7144e425
Apperley, J.F.
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Huguet, F.
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Pitini, V.
cd4cd6b1-493f-4b8f-a914-537b9e5e12dd
Gardembas, M.
5c9c797e-0132-4413-8dbd-0935c524efca
Ross, D.M.
924baec1-2805-46bb-a15c-294c7434ce96
Forrest, D.
1f438101-e1cb-4ca9-8517-6da7da2094b2
Genet, P.
2f6fd379-a244-404a-b414-2a2df370fa5c
Rousselot, P.
a318d5f8-e6c6-4001-9358-e9153502e0df
Patton, N.
62e7d76f-8dd1-4155-b352-f49941f5dca9
Smith, G.
adee1144-0354-42ec-8b09-86d5974969ea
Dunbar, C.E.
43b92f52-1a24-4c23-95ee-0d5f3d408c25
Ito, S.
e183958d-f7bd-441f-809e-423b59f71ce1
Aguiar, R.C.T.
916b0c2d-c08e-4646-9797-1f30c81a95f2
Odenike, O.
ffb11583-aa21-47e3-995e-3151f886b30d
Gimelfarb, A.
892cf87e-bd4c-4d24-9b64-1614ba0c96b8
Cross, N.C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4
Seymour, J.F.
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Cheah, C.Y., Burbury, K., Apperley, J.F., Huguet, F., Pitini, V., Gardembas, M., Ross, D.M., Forrest, D., Genet, P., Rousselot, P., Patton, N., Smith, G., Dunbar, C.E., Ito, S., Aguiar, R.C.T., Odenike, O., Gimelfarb, A., Cross, N.C.P. and Seymour, J.F. (2014) Patients with myeloid malignancies bearing PDGFRB fusion genes achieve durable long term remissions with imatinib. Blood, 123 (23), 3574-3577. (doi:10.1182/blood-2014-02-555607). (PMID:24687085)

Record type: Article

Abstract

Myeloid neoplasms and eosinophilia with rearrangements of PDGFRB are uncommon Philadelphia negative myeloproliferative neoplasms. Patients are typically male, with morphologic features of a Ph- chronic myeloproliferative syndrome or chronic myelomonocytic leukemia with eosinophilia. Reciprocal translocations involving PDGFRB result in fusion genes with constitutively activated receptor tyrosine kinase sensitive to inhibition with imatinib. We present an updated and expanded analysis of a cohort of 26 such patients treated with imatinib. After a median follow-up of 10.2 (range 1.8 - 17) years, the 10-year overall survival rate was 90% (95%CI 64-97%); after median imatinib duration of 6.6 (range 0.1 - 12) years the 6-year progression-free survival rate was 88% (95% CI; 65-96%). 96% patients responded; no patients who achieved a complete cytogenetic (n=13) or molecular (n=8) remission lost their response or progressed to blast crisis. Imatinib is well tolerated and achieves excellent long-term responses in patients with PDGFRB rearrangements.

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More information

Accepted/In Press date: 31 March 2014
Published date: June 2014
Organisations: Human Development & Health

Identifiers

Local EPrints ID: 364167
URI: http://eprints.soton.ac.uk/id/eprint/364167
ISSN: 0006-4971
PURE UUID: 02b386f0-0ff5-4aaf-9eea-1332b0ad396b
ORCID for N.C.P. Cross: ORCID iD orcid.org/0000-0001-5481-2555

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Date deposited: 07 Apr 2014 16:16
Last modified: 15 Mar 2024 03:11

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Contributors

Author: C.Y. Cheah
Author: K. Burbury
Author: J.F. Apperley
Author: F. Huguet
Author: V. Pitini
Author: M. Gardembas
Author: D.M. Ross
Author: D. Forrest
Author: P. Genet
Author: P. Rousselot
Author: N. Patton
Author: G. Smith
Author: C.E. Dunbar
Author: S. Ito
Author: R.C.T. Aguiar
Author: O. Odenike
Author: A. Gimelfarb
Author: N.C.P. Cross ORCID iD
Author: J.F. Seymour

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