Tyrosine kinase fusion genes in chronic myeloproliferative diseases
Tyrosine kinase fusion genes in chronic myeloproliferative diseases
With the exception of chronic myeloid leukemia (CML), chronic myeloproliferative disorders (CMPDs) are a heterogeneous spectrum of conditions for which the molecular pathogenesis is not well understood. Most cases have a normal or aneuploid karyotype, but a minority present with a reciprocal translocation that disrupts specific tyrosine kinase genes, most commonly PDGFRB or FGFR1. These translocations result in the production of constitutively active tyrosine kinase fusion proteins that deregulate hemopoiesis in a manner analogous to BCR-ABL. With the advent of targeted signal transduction therapy, an accurate clinical and molecular diagnosis of CMPDs has become increasingly important. Currently, patients with PDGFRB or ABL fusion genes are candidates for treatment with Imatinib (STI571), but it is likely that alternative strategies will be necessary for the treatment of most other patients.
tyrosine kinase, myeloproliferative disorders, ABL, FGFR1, PDGFRB, JAK2
1207-1212
Cross, N.C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4
Reiter, A.
8fc082eb-6b46-4412-86b2-f4c7669f0650
2002
Cross, N.C.P.
f87650da-b908-4a34-b31b-d62c5f186fe4
Reiter, A.
8fc082eb-6b46-4412-86b2-f4c7669f0650
Cross, N.C.P. and Reiter, A.
(2002)
Tyrosine kinase fusion genes in chronic myeloproliferative diseases.
Leukemia, 16 (7), .
(doi:10.1038/sj.leu.2402556).
Abstract
With the exception of chronic myeloid leukemia (CML), chronic myeloproliferative disorders (CMPDs) are a heterogeneous spectrum of conditions for which the molecular pathogenesis is not well understood. Most cases have a normal or aneuploid karyotype, but a minority present with a reciprocal translocation that disrupts specific tyrosine kinase genes, most commonly PDGFRB or FGFR1. These translocations result in the production of constitutively active tyrosine kinase fusion proteins that deregulate hemopoiesis in a manner analogous to BCR-ABL. With the advent of targeted signal transduction therapy, an accurate clinical and molecular diagnosis of CMPDs has become increasingly important. Currently, patients with PDGFRB or ABL fusion genes are candidates for treatment with Imatinib (STI571), but it is likely that alternative strategies will be necessary for the treatment of most other patients.
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Published date: 2002
Keywords:
tyrosine kinase, myeloproliferative disorders, ABL, FGFR1, PDGFRB, JAK2
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Local EPrints ID: 24669
URI: http://eprints.soton.ac.uk/id/eprint/24669
ISSN: 0887-6924
PURE UUID: b50e27b9-ba18-470f-a9a5-012784677e5e
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Date deposited: 03 Apr 2006
Last modified: 16 Mar 2024 03:23
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Author:
A. Reiter
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