Mechanisms of Thrombogenesis

  • Anna Falanga
  • Laura Russo
  • Marina Marchetti
Part of the Hematologic Malignancies book series (HEMATOLOGIC)


Thrombosis is a leading cause of morbidity and mortality in patients with polycythemia vera (PV) and essential thrombocythemia (ET). A number of mechanisms have been proposed to play a role in the pathogenesis of the acquired thrombophilic state in these diseases, including red blood cell, platelet, and leukocyte abnormalities. Published data demonstrate that neutrophil activation occurs in ET and PV patients in parallel with the appearance of laboratory signs of hemostatic system activation, suggesting an involvement of these cells in the pathogenesis of the thrombotic predisposition of these subjects. Recently, an increase in plasma procoagulant microparticles and the occurrence of an acquired activated protein C resistance have been identified as other two possible mechanisms of systemic hypercoagulability. The acquired point mutation in the pseudokinase domain of Janus kinase 2 (JAK2V617F) in these disorders is under evaluation as a risk factor for thrombosis. JAK2V617F is variably associated with thrombosis and, more consistently, with elevations in blood cell counts. A clear link appears to exist between leukocytosis, JAK2V617F, and the hemostatic system abnormalities underlying the activation of blood coagulation in patients with Bcl-negative myeloproliferative neoplasms.


Polycythemia Vera Essential Thrombocythemia JAK2V617F Mutation Polycythemia Vera Patient Acute Chest Syndrome 
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© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  1. 1.Division of Immunohematology and Transfusion MedicineOspedali Riuniti di BergamoBergamoItaly

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