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Vol. 119, No. 4, 2008
Issue release date: July 2008
Section title: Molecular Drug Targets in Myeloproliferative Neoplasms
Acta Haematol 2008;119:199–206
(DOI:10.1159/000140631)

Fibroblast Growth Factor Receptor and Platelet-Derived Growth Factor Receptor Abnormalities in Eosinophilic Myeloproliferative Disorders

Cross N.C.P. · Reiter A.
aWessex Regional Genetics Laboratory, University of Southampton, Salisbury District Hospital, Salisbury, UK; bIII. Medizinische Universitätsklinik, Medizinische Fakultät Mannheim der Universität Heidelberg, Mannheim, Germany

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Article / Publication Details

First-Page Preview
Abstract of Molecular Drug Targets in Myeloproliferative Neoplasms

Published online: 6/20/2008

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA

Abstract

Rearrangements of the genes encoding the fibroblast growth factor receptor 1 (FGFR1) and platelet-derived growth factor receptors (PDGFR) α or β receptor tyrosine kinases are found in a rare but important subset of patients with atypical myeloproliferative disorders that are usually but not always associated with eosinophilia. Chromosomal translocations or other rearrangements at 8p11–12, 4q12 or 5q31–33 give rise to diverse fusion genes encoding chimaeric proteins with constitutive transforming activity. There is considerable molecular heterogeneity with 8 partner genes currently known for FGFR1, 6 for PDGFRA and 17 for PDGFRB. The vast majority of patients with PDGFRA or PDGFRB fusions achieve rapid and durable complete haematological and molecular responses to sustained imatinib therapy. A key ongoing challenge is to define the molecular pathogenesis of the great majority of atypical myeloproliferative disorders for whom the causative lesion remains unknown, since very few of these cases gain any benefit from imatinib or other second-generation inhibitors.


Article / Publication Details

First-Page Preview
Abstract of Molecular Drug Targets in Myeloproliferative Neoplasms

Published online: 6/20/2008

Number of Print Pages: 8
Number of Figures: 2
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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