Intraplatelet levels of vWF:Ag and fibrinogen in myeloproliferative disorders.
Several platelet function abnormalities have been described in the myeloproliferative syndromes. We have measured the intraplatelet vWF:Ag and fibrinogen (FI) in the platelet lysates by Laurell technique in 11 patients with polycythemia vera (PV), 10 with essential thrombocythemia (ET), 14 with chronic myelocytic leukaemia (CML) and 3 with myelofibrosis (MF) and these results were correlated with platelet function abnormalities. Decreased intraplatelet levels of vWF:Ag and FI were found in all the patients with ET and MF, in 8 out of 11 PV and 3 out of 14 CML. A statistical significant correlation was observed between the intraplatelet levels of vWF:Ag and FI in the control group and in CML and PV, but no correlation was found in ET and MF. No correlation was observed between the plasmatic and the intraplatelet levels of vWF:Ag and FI in any group. Evidences of platelet activation (spontaneous platelet aggregation or circulating platelet aggregates) were observed in 40% of the cases with ET and PV, and all these cases had low intraplatelet levels of both antigens. None of the cases with MF had evidences of platelet activation and 2 out of 14 patients with CML had platelet activation. The deficiency of the dense bodies was less frequent than the depletion of the alpha granules (5 out of 11 PV, 4 out of 10 ET, 6 out of 14 CML and 2 out of 3 MF). The low intraplatelet contents of vWF: Ag and FI, more frequently observed in ET and PV, may be the result of platelet activation and in vivo release, but megakaryocyte dysfunction is more likely in myelofibrosis.
D000941:Antigens / Q000032:analysis*; D001792:Blood Platelets / Q000276:immunology / Q000502:physiology*; D005340:Fibrinogen / Q000032:analysis*; D006801:Humans; D007951:Leukemia, Myeloid / Q000097:blood; D009196:Myeloproliferative Disorders / Q000097:blood* / Q000276:immunology; D011087:Polycythemia Vera / Q000097:blood; D055728:Primary Myelofibrosis / Q000097:blood; D012016:Reference Values; D013920:Thrombocythemia, Essential / Q000097:blood; D014841:von Willebrand Factor / Q000276:immunology*
D000941:Antigens; C029417:Von Willebrand antigen; D014841:von Willebrand Factor; D005340:Fibrinogen