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Vol. 107, No. 2, 2002
Issue release date: March 2002
Section title: Paper
Acta Haematol 2002;107:113–122
(DOI:10.1159/000046641)

Myeloproliferative Disorders with Translocations of Chromosome 5q31–35: Role of the Platelet-Derived Growth Factor Receptor Beta

Steer E.J. · Cross N.C.P.
Wessex Regional Genetics Laboratory, Salisbury, andHuman Genetics Division, University of Southampton School of Medicine, Southampton, UK

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Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 3/27/2002

Number of Print Pages: 10
Number of Figures: 1
Number of Tables: 3

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

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

Abstract

Acquired reciprocal chromosomal translocations that involve chromosome bands 5q31–33 are associated with a significant minority of patients with BCR-ABL-negative chronic myeloid leukemias. The most common abnormality is the t(5;12)(q33;p13), which fuses the ETV6/TEL gene to the platelet-derived growth factor receptor-β (PDGFRB), a receptor tyrosine kinase that maps to 5q33. PDGFRB is disrupted by other translocations and to date four additional partner genes (H4, HIP1, CEV14 and Rab5) have been reported. Clinically, most patients present with a myeloproliferative disorder (MPD) with eosinophilia, eosinophilic leukemia or chronic myelomonocytic leukemia and thus fall into the broader category of myeloproliferative disorders/myelodysplastic syndromes (MPD/MDS). With the advent of targeted signal transduction therapy, patients with rearrangement of PDGFRB might be better classified as a distinct subgroup of MPD/MDS.


  

Author Contacts

Prof. N.C.P. Cross
Wessex Regional Genetics Laboratory
Salisbury District Hospital
Salisbury SP2 8BJ (UK)
Tel. +44 1722 429080, Fax +44 1722 338095, E-Mail ncpc@soton.ac.uk

  

Article Information

Number of Print Pages : 10
Number of Figures : 1, Number of Tables : 3, Number of References : 77

  

Publication Details

Acta Haematologica
Founded 1948
Affiliated with Molecular Biology of Hematopoiesis Symposium

Vol. 107, No. 2, Year 2002 (Cover Date: Released March 2002)

Journal Editor: I. Ben-Bassat, Tel Hashomer
ISSN: 0001–5792 (print), 1421–9662 (Online)

For additional information: http://www.karger.com/journals/aha


Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 3/27/2002

Number of Print Pages: 10
Number of Figures: 1
Number of Tables: 3

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

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


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