Identification of a MY018A-PDGFRB fusion gene in an eosinophilia-associated atypical myeloproliferative neoplasm with a t(5;17)(q33-34;q11.2)
Identification of a MY018A-PDGFRB fusion gene in an eosinophilia-associated atypical myeloproliferative neoplasm with a t(5;17)(q33-34;q11.2)
Chromosomal aberrations of 5q31-33 associated with rearrangements of the platelet-derived growth factor receptor beta (PDGFRB) gene are rare but recurrent in patients with eosinophilia-associated atypical myeloproliferative neoplasms (Eos-MPNs). We used a DNA-based long-distance inverse PCR (LDI-PCR) to identify a new MYO18A-PDGFRB fusion gene in an Eos-MPN with associated t(5;17)(q33-34;q11.2). MYO18A is the fourth partner gene after BCR, ETV6 and SPTBN1 that fuses to more than one tyrosine kinase gene. Treatment with imatinib (400 mg/day) led to rapid and sustained complete hematologic, cytogenetic and molecular remission. Patients with PDGFRB fusions genes are excellent candidates for treatment with imatinib; complete cytogenetic and even molecular remissions are common while primary or secondary resistance seems to be very rare
treatment, kinase, secondary, neoplasms, gene, imatinib, platelet-derived growth factor, germany, growth, tyrosine, genes, resistance, patients
179-183
Walz, Christoph
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Haferlach, Claudia
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Hänel, Annette
d3255b03-6c94-444d-8767-7fbb5999f4fd
Metzgeroth, Georgia
611ec46d-9a11-4e24-ae0f-5ac19dfd0237
Erben, Phillip
30ce462f-e220-4cce-a805-792cdbf27b0a
Gosenca, Darko
0a32fea9-fcac-4e5c-83a3-65c8e99ea704
Hochhaus, Andreas
b37b9b7d-85ff-455e-994d-fcc2adf94088
Cross, Nicholas C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4
Reiter, Andreas
ffa23e84-4a13-4cb5-aaf0-3fafe25dbede
February 2009
Walz, Christoph
b8d235ac-2a38-41e7-a22f-e0bb78719b19
Haferlach, Claudia
0bf0896a-7b9c-451a-9e64-935ecdd7aa15
Hänel, Annette
d3255b03-6c94-444d-8767-7fbb5999f4fd
Metzgeroth, Georgia
611ec46d-9a11-4e24-ae0f-5ac19dfd0237
Erben, Phillip
30ce462f-e220-4cce-a805-792cdbf27b0a
Gosenca, Darko
0a32fea9-fcac-4e5c-83a3-65c8e99ea704
Hochhaus, Andreas
b37b9b7d-85ff-455e-994d-fcc2adf94088
Cross, Nicholas C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4
Reiter, Andreas
ffa23e84-4a13-4cb5-aaf0-3fafe25dbede
Walz, Christoph, Haferlach, Claudia, Hänel, Annette, Metzgeroth, Georgia, Erben, Phillip, Gosenca, Darko, Hochhaus, Andreas, Cross, Nicholas C.P. and Reiter, Andreas
(2009)
Identification of a MY018A-PDGFRB fusion gene in an eosinophilia-associated atypical myeloproliferative neoplasm with a t(5;17)(q33-34;q11.2).
Genes, Chromosomes and Cancer, 48 (2), .
(doi:10.1002/gcc.20629).
Abstract
Chromosomal aberrations of 5q31-33 associated with rearrangements of the platelet-derived growth factor receptor beta (PDGFRB) gene are rare but recurrent in patients with eosinophilia-associated atypical myeloproliferative neoplasms (Eos-MPNs). We used a DNA-based long-distance inverse PCR (LDI-PCR) to identify a new MYO18A-PDGFRB fusion gene in an Eos-MPN with associated t(5;17)(q33-34;q11.2). MYO18A is the fourth partner gene after BCR, ETV6 and SPTBN1 that fuses to more than one tyrosine kinase gene. Treatment with imatinib (400 mg/day) led to rapid and sustained complete hematologic, cytogenetic and molecular remission. Patients with PDGFRB fusions genes are excellent candidates for treatment with imatinib; complete cytogenetic and even molecular remissions are common while primary or secondary resistance seems to be very rare
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Published date: February 2009
Keywords:
treatment, kinase, secondary, neoplasms, gene, imatinib, platelet-derived growth factor, germany, growth, tyrosine, genes, resistance, patients
Organisations:
Human Genetics
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Local EPrints ID: 69715
URI: http://eprints.soton.ac.uk/id/eprint/69715
ISSN: 1045-2257
PURE UUID: 82b0e7f9-fd8e-4127-b6b2-7f52236d198f
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Date deposited: 27 Nov 2009
Last modified: 14 Mar 2024 02:46
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Author:
Christoph Walz
Author:
Claudia Haferlach
Author:
Annette Hänel
Author:
Georgia Metzgeroth
Author:
Phillip Erben
Author:
Darko Gosenca
Author:
Andreas Hochhaus
Author:
Andreas Reiter
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