PMID: 15378946Jan 1, 1994Paper

Platelet abnormalities in idiopathic myelofibrosis: functional, biochemical and immunomorphological correlations

Haematologica
P LeoniG Biagini

Abstract

An extensive study of platelet function was performed on 18 consecutive patients affected by idiopathic myelofibrosis (IM). Clinical hematological and morphofunctional parameters were studied in IM patients and control subjects. Platelet tests, ultrastructural data, immunocytochemical von Willebrand factor detection, freeze fracturing results and free cytosolic calcium level were evaluated. Bleeding time was frequently found to be prolonged, but it never reached levels which could give any cause for concern. Aggregation by ADP, collagen and epinephrine was always altered, sometimes profoundly; on the contrary, agglutination by ristocetin was almost always normal, albeit occasionally increased. Plasma beta-TG and PF4 levels were found to be elevated in 11 and 12 patients, respectively. This indicated an abnormal release from platelet alpha-granules. Depletion of alpha-granules was also confirmed by the intraplatelet von Willebrand factor (vWF) labelling with colloidal gold particles bound to polyclonal antibodies against human vWF. In fact: 1) the number of positive alpha-granules/microm2 and per single platelet was reduced; 2) the intensity of the immunocytochemical reaction for single positive alpha-granules and for each plate...Continue Reading

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