The University of Southampton
University of Southampton Institutional Repository

Myeloproliferative disorders

Myeloproliferative disorders
Myeloproliferative disorders
The myeloproliferative disorders (MPDs) are a group of pre-leukaemic disorders characterized by proliferation of one or more lineages of the myelo-erythroid series. Unlike the Philadelphia chromosome in chronic myeloid leukaemia, there is no pathognomonic chromosomal abnormality associated with the MPDs. Chromosomal abnormalities are seen in 30–40% of patients with polycythaemia vera (PV) and idiopathic myelofibrosis (IMF) and seem to indicate a poor prognosis. On the other hand, chromosomal abnormalities are rare in essential thrombocythaemia. Consistent acquired changes seen at diagnosis include deletion of the long arm of chromosome 20, del(13q), trisomy 8 and 9 and duplication of parts of 1q. Furthermore del(20q), trisomy 8 and dupl(lq) all arise in multipotent progenitor cells. Molecular mapping of 20q deletions and, to some extent, 13q deletions has identified a number of candidate target genes, although no mutations have yet been found. Finally, translocations associated with the rare 8p11 myeloproliferative syndrome and other atypical myeloproliferative disorders have permitted the identification of a number of novel fusion proteins involving fibroblast growth factor receptor-1.
myeloproliferative disorders, chromosomal abnormalities, deletions, amplifications, translocations
1521-6926
531-551
Bench, Anthony J.
90d97689-67df-4617-bf72-4d91f1995a1c
Cross, Nicholas C.
f87650da-b908-4a34-b31b-d62c5f186fe4
Huntly, Brian J.P.
287595f9-eab5-4914-b1fe-78fadc07cae2
Nacheva, Elisabeth P.
3052e0f3-bd51-463e-a4e0-5e69513e6ab2
Green, Anthony R.
2723b7ae-f4d6-436b-ab71-fad03823b25c
Bench, Anthony J.
90d97689-67df-4617-bf72-4d91f1995a1c
Cross, Nicholas C.
f87650da-b908-4a34-b31b-d62c5f186fe4
Huntly, Brian J.P.
287595f9-eab5-4914-b1fe-78fadc07cae2
Nacheva, Elisabeth P.
3052e0f3-bd51-463e-a4e0-5e69513e6ab2
Green, Anthony R.
2723b7ae-f4d6-436b-ab71-fad03823b25c

Bench, Anthony J., Cross, Nicholas C., Huntly, Brian J.P., Nacheva, Elisabeth P. and Green, Anthony R. (2001) Myeloproliferative disorders. Best Practice & Research: Clinical Haematology, 14 (3), 531-551. (doi:10.1053/beha.2001.0153).

Record type: Article

Abstract

The myeloproliferative disorders (MPDs) are a group of pre-leukaemic disorders characterized by proliferation of one or more lineages of the myelo-erythroid series. Unlike the Philadelphia chromosome in chronic myeloid leukaemia, there is no pathognomonic chromosomal abnormality associated with the MPDs. Chromosomal abnormalities are seen in 30–40% of patients with polycythaemia vera (PV) and idiopathic myelofibrosis (IMF) and seem to indicate a poor prognosis. On the other hand, chromosomal abnormalities are rare in essential thrombocythaemia. Consistent acquired changes seen at diagnosis include deletion of the long arm of chromosome 20, del(13q), trisomy 8 and 9 and duplication of parts of 1q. Furthermore del(20q), trisomy 8 and dupl(lq) all arise in multipotent progenitor cells. Molecular mapping of 20q deletions and, to some extent, 13q deletions has identified a number of candidate target genes, although no mutations have yet been found. Finally, translocations associated with the rare 8p11 myeloproliferative syndrome and other atypical myeloproliferative disorders have permitted the identification of a number of novel fusion proteins involving fibroblast growth factor receptor-1.

Full text not available from this repository.

More information

Published date: 2001
Additional Information: Regular Article
Keywords: myeloproliferative disorders, chromosomal abnormalities, deletions, amplifications, translocations

Identifiers

Local EPrints ID: 24627
URI: https://eprints.soton.ac.uk/id/eprint/24627
ISSN: 1521-6926
PURE UUID: eb1e4317-b509-411f-93a4-61ee4c8cdee8
ORCID for Nicholas C. Cross: ORCID iD orcid.org/0000-0001-5481-2555

Catalogue record

Date deposited: 04 Apr 2006
Last modified: 06 Oct 2018 00:36

Export record

Altmetrics

Contributors

Author: Anthony J. Bench
Author: Brian J.P. Huntly
Author: Elisabeth P. Nacheva
Author: Anthony R. Green

University divisions

Download statistics

Downloads from ePrints over the past year. Other digital versions may also be available to download e.g. from the publisher's website.

View more statistics

Atom RSS 1.0 RSS 2.0

Contact ePrints Soton: eprints@soton.ac.uk

ePrints Soton supports OAI 2.0 with a base URL of https://eprints.soton.ac.uk/cgi/oai2

This repository has been built using EPrints software, developed at the University of Southampton, but available to everyone to use.

We use cookies to ensure that we give you the best experience on our website. If you continue without changing your settings, we will assume that you are happy to receive cookies on the University of Southampton website.

×