Genetic and epigenetic alterations of myeloproliferative disorders

The classical BCR–ABL negative myeloproliferative neoplasms (MPN) polycythemia vera, essential thrombocythemia, and primary myelofibrosis are clonal hematopoietic disorders characterized by excessive production of terminally differentiated myeloid cells. In MPN patients, the disease can progress to...

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Bibliographic Details
Published in:International journal of hematology 2013-02, Vol.97 (2), p.183-197
Main Authors: Milosevic, Jelena D., Kralovics, Robert
Format: Article
Language:English
Subjects:
Abl protein
Adaptor Proteins, Signal Transducing
Chromosome Aberrations
Dioxygenases
Disease Progression
DNA (Cytosine-5-)-Methyltransferases - genetics
DNA Methyltransferase 3A
DNA-Binding Proteins - genetics
Epigenesis, Genetic
Hematology
Humans
Intracellular Signaling Peptides and Proteins
Isocitrate Dehydrogenase - genetics
Janus Kinase 2 - genetics
Medicine
Medicine & Public Health
Mutation
Myeloproliferative Disorders - genetics
Oncology
Polycomb Repressive Complex 2 - genetics
Progress in Hematology
Proteins - genetics
Proto-Oncogene Proteins - genetics
Proto-Oncogene Proteins c-cbl - genetics
Receptors, Thrombopoietin - genetics
Repressor Proteins - genetics
RNA Splicing
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Description
Summary:The classical BCR–ABL negative myeloproliferative neoplasms (MPN) polycythemia vera, essential thrombocythemia, and primary myelofibrosis are clonal hematopoietic disorders characterized by excessive production of terminally differentiated myeloid cells. In MPN patients, the disease can progress to secondary myelofibrosis or acute myeloid leukemia. Clonal hematopoiesis, disease phenotype, and progression are caused by somatically acquired genetic lesions of genes involved in cytokine signaling, RNA splicing, as well as epigenetic regulation. This review provides an overview of point mutations and cytogenetic lesions associated with MPN and addresses the role of these somatic lesions in MPN disease progression.
ISSN:0925-5710
1865-3774
DOI:10.1007/s12185-012-1235-2