The myeloproliferative disorders comprise several clonal hematologicdiseases that are thought to arise from a transformation ina hematopoietic stem cell. The main clinical features of thesediseases are the overproduction of mature, functional bloodcells and a long clinical course. Chronic myeloid leukemia (notdiscussed in detail here) is a myeloproliferative disorder thatis defined by its causative molecular lesion, the BCR-ABL fusiongene, which most commonly results from the Philadelphia translocation(Ph).1 The three main Ph-negative myeloproliferative disorders polycythemia vera, essential thrombocythemia, and idiopathicmyelofibrosis are the focus of this article. Less commonconditions 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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