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Cellular and Molecular Life Sciences CMLS

, Volume 63, Issue 24, pp 2939–2953 | Cite as

Oncogenic mechanisms in myeloproliferative disorders

  • F. Delhommeau
  • D. F. Pisani
  • C. James
  • N. Casadevall
  • S. Constantinescu
  • W. VainchenkerEmail author
Review

Abstract.

Myeloproliferative disorders (MPDs) are clonal haematopoietic malignancies involving the abnormal proliferation of myeloid lineages. The World Health Organisation (WHO) classification of haematopoietic malignancies distinguishes MPDs from myelodysplastic/ myeloproliferative disorders and systemic mastocytosis. These malignancies frequently involve constitutive tyrosine kinase activity, resulting from either oncogenic fusion protein production or from point mutations. Chronic myelogenous leukaemia is the model used for studies of the consequences of such molecular defects. However, the heterogeneity of the clinical course of MPDs should be seen in a more rationale conceptual framework, including the many molecular events associated with these diseases. This review focuses on the various tyrosine kinase-related molecular mechanisms underlying both MPDs and rare diseases with myeloproliferative features. We pay particular attention to the newly identified JAK2 V617F mutation in polycythaemia vera, essential thrombocythaemia and idiopathic myelofibrosis and deal with disease heterogeneity and putative additional molecular mechanisms.

Keywords.

Myeloproliferative disorders tyrosine kinase BCR/ABL signal transduction JAK2 leukaemogenesis 

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Copyright information

© Birkhäuser Verlag, Basel 2006

Authors and Affiliations

  • F. Delhommeau
    • 1
    • 2
  • D. F. Pisani
    • 1
  • C. James
    • 1
    • 3
  • N. Casadevall
    • 1
    • 2
  • S. Constantinescu
    • 4
  • W. Vainchenker
    • 1
    Email author
  1. 1.INSERM, U790Université Paris Sud, Institut Gustave RoussyVillejuifFrance
  2. 2.AP-HP, Laboratoire d’Hématologie, Hôpital Saint-Antoine & Hôpital Hôtel-DieuUniversité Pierre et Marie CurieParisFrance
  3. 3.INSERM E217Université Victor Ségalen Bordeaux 2BordeauxFrance
  4. 4.Ludwig Institute for Cancer Research and Christian de Duve Institute of Cellular Pathology and MEXP UnitUniversité Catholique de LouvainBrusselsBelgium

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