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Review Article
Mechanisms of Disease
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Volume 355:2452-2466 December 7, 2006 Number 23
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The Myeloproliferative Disorders
Peter J. Campbell, F.R.A.C.P., F.R.C.P.A., and Anthony R. Green, F.R.C.Path., F.Med.Sci.

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The myeloproliferative disorders comprise several clonal hematologic diseases that are thought to arise from a transformation in a hematopoietic stem cell. The main clinical features of these diseases are the overproduction of mature, functional blood cells and a long clinical course. Chronic myeloid leukemia (not discussed in detail here) is a myeloproliferative disorder that is defined by its causative molecular lesion, the BCR-ABL fusion gene, which most commonly results from the Philadelphia translocation (Ph).1 The three main Ph-negative myeloproliferative disorders — polycythemia vera, essential thrombocythemia, and idiopathic myelofibrosis — are the focus of this article. Less common conditions that are . . . [Full Text of this Article]

Pathogenetic Features

Clues to Molecular Mechanisms

The JAK2 V617F Mutation

Pathophysiological Features

Genetic Marker

Mouse Models

Stem-Cell Biology

Cooperating Mutations

Homozygosity for V617F

Signaling Pathways

Disease Evolution

Molecular Classification

Diagnosis

Treatment


Source Information

From the Department of Haematology, Cambridge Institute for Medical Research, University of Cambridge, Cambridge, United Kingdom.

Address reprint requests to Dr. Green at the Department of Haematology, Cambridge Institute for Medical Research, Hills Rd., Cambridge CB2 2XY, United Kingdom, or at arg1000@cam.ac.uk.


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