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Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis.

Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis. Research Abstract Details 

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  • Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis. Abstract Text:

    kais husseinKais Hussein,kai brakensiekKai Brakensiek,guntram buescheGuntram Buesche,thomas buhrThomas Buhr,birgitt wieseBirgitt Wiese,hans kreipeHans Kreipe,oliver bockOliver Bock,kais husseinKais Hussein,kai brakensiekKai Brakensiek,guntram buescheGuntram Buesche,thomas buhrThomas Buhr,birgitt wieseBirgitt Wiese,hans kreipeHans Kreipe,oliver bockOliver Bock,

    Atypical megakaryocytes provide the histomorphological hallmark of all Philadelphia-chromosome negative chronic myeloproliferative disorder (Ph(-) CMPD) subtypes and have not been studied so far for the JAK2(V617F) mutation. The mutant gene dosage was determined in isolated megakaryocytes from 68 cases of JAK2(+)/Ph(-) CMPD by a pyrosequencing assay. Megakaryocytes from essential thrombocythemia (ET) showed significantly lower levels of mutated JAK2 alleles compared to patients with chronic idiopathic myelofibrosis (cIMF) with manifest fibrosis and polycythemia vera (PV) but not to prefibrotic cIMF. Solely, ET JAK2V617F in megakaryocytes is associated with a PV-like phenotype, and at least in one patient, the JAK2 mutation was exclusively acquired within the megakaryocytic lineage. The overt differences between prefibrotic and fibrotic cIMF suggested a causative role of the gene dosage of mutant JAK2 in fibrotic progression. Megakaryocyte analysis of a follow-up of eight individual cases with sequential biopsies, however, showed that progression to homozygosity of V617F mutated JAK2 and onset of manifest fibrosis appeared to be independent events. We conclude that megakaryocytes might be the predominant or even the exclusive lineage that acquires the JAK2(V617F) mutation in ET and that the JAK2(V617F) evolution to higher gene dosages represents a dynamic and complex process substantially involving megakaryocytes.

    Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis. Publishing Authors By Initials

    k husseinK Hussein,k brakensiekK Brakensiek,g buescheG Buesche,t buhrT Buhr,b wieseB Wiese,h kreipeH Kreipe,o bockO Bock,k husseinK Hussein,k brakensiekK Brakensiek,g buescheG Buesche,t buhrT Buhr,b wieseB Wiese,h kreipeH Kreipe,o bockO Bock,

    For similar abstracts research abstracts see: abstracts research

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    Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis. Journal Published:

    PUBLICATION TYPE: Research Support, Non-U.S. Gov

    Journal: Annals of hematology

    VOLUME: 86

    Page Numbers: 245-53

    Journal Abbreviation: Ann. Hematol.

    ISSN: 1432-0584

    DAY: 30

    MONTH: 01

    YEAR: 2007

    Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis. Information

    Number of References:

    LANGUAGE: eng

    NlmUniqueID: 9107334

    Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis. Keywords Mesh Terms:

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    Grant and Affiliation Information for Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis.

    AFFILIATION: Institute of Pathology, Hannover Medical School, Hannover, Germany.

    Country: Germany

    Germany Research PublicationGermany Research Publication

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    MEDLINETA: Ann Hematol

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    Different involvement of the megakaryocytic lineage by the JAK2 V617F mutation in Polycythemia vera, essential thrombocythemia and chronic idiopathic myelofibrosis Related Publications

     

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