Original Article

Annals of Hematology

, Volume 87, Issue 4, pp 269-276

Increased platelet, leukocyte, and coagulation activation in primary myelofibrosis

  • Alberto Alvarez-LarránAffiliated withHematology Department, Hospital Clínic, IDIBAPS, University of Barcelona
  • , Eduardo Arellano-RodrigoAffiliated withHematology Department, Hospital Clínic, IDIBAPS, University of Barcelona
  • , Juan Carlos ReverterAffiliated withHemotherapy and Hemostasis Department, Hospital Clínic, IDIBAPS, University of Barcelona
  • , Abel DomingoAffiliated withHematology Department, Hospital Clínic, IDIBAPS, University of Barcelona
  • , Neus VillamorAffiliated withHematopathology Department, Hospital Clínic, IDIBAPS, University of Barcelona
  • , Dolors ColomerAffiliated withHematopathology Department, Hospital Clínic, IDIBAPS, University of Barcelona
  • , Francisco CervantesAffiliated withHematology Department, Hospital Clínic, IDIBAPS, University of Barcelona Email author 

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Abstract

Platelet and leukocyte activation has been demonstrated in polycythemia vera (PV) and essential thrombocythemia (ET), but such information is limited in primary myelofibrosis (PMF). Platelet, leukocyte, endothelial, and coagulation activation status was assessed in 26 PMF patients and compared with data from 22 age- and sex-matched healthy individuals. Study included flow cytometry assessment of platelet P-selectin expression [at baseline and after adenosine diphosphate (ADP), thrombin and arachidonic acid stimulation], platelet–neutrophil and platelet–monocyte complexes, and CD11b expression in neutrophils and monocytes. Additionally, soluble P-selectin, sCD40L, tissue factor, thrombomodulin, prothrombin fragment 1 + 2 (F1 + 2), and D-dimer were measured by enzyme-linked immunosorbent assays. The above parameters were correlated with the patients’ clinical data and presence of the JAK2 V617F mutation. Compared with controls, PMF patients had increased baseline platelet activation, as shown by significantly higher levels of soluble and platelet P-selectin expression, and also higher percentages of platelet–monocyte complexes. Neutrophil and monocyte CD11b expression was significantly higher in patients with the JAK2 mutation than in those with wild-type allele or the controls. Endothelial and coagulation activation, as demonstrated by increased plasma levels of thrombomodulin and F1 + 2, was also found in PMF, with patients with the JAK2 mutation showing significantly higher values of F1 + 2 than those with wild-type allele. In conclusion, PMF patients have platelet, leukocyte, endothelial, and coagulation activation similar to that in PV and ET. CD11b overexpression and F1 + 2 are correlated with the presence of the JAK2 mutation.

Keywords

Primary myelofibrosis Platelet activation Leukocyte activation Coagulation activation JAK2 mutation