Abstract

VEGA, Sara et al. Estudio ultraestructural de las plaquetas de pacientes con hemoglobinuria paroxística nocturnaUltrastructural study of platelets patients with paroxysmal nocturnal hemoglobinuria. Rev. invest. clín. [online]. 2006, vol.58, n.5, pp.475-486. ISSN 2564-8896.

Introduction. Paroxysmal nocturnal hemoglobinuria (PNH) is a hemolytic anemia characterized by intravascular hemolysis, cytopenias and venous thrombosis. Previous studies in patients with PNH have shown platelet abnormalities; however, their association with the clinical development of the sickness has not still been determined. Material and methods. In this study, we compared the morphology and distribution pattern of actin, myosin, tubulin and p-selectin in resting and activated platelets from 22 PNH patients and healthy donors by transmission electron microscopy and immunofluorescence. Results. The PNH platelet ultrastructure of resting and activated with different agonists (ADP, collagen and thrombin) showed morphological changes which suggested the presence of circulating platelets. The developed structures during the adhesion process (filopodia and lamellipodia formation), as well as the pattern distribution of actin, myosin, tubulin and p-selectin in PNH platelets were not modified in relation to control platelets. Conclusion. Morphological changes in resting platelets were related with p-selectin expression suggesting its determination as thrombosis indicator.

Keywords : Platelets; Activation; PNH; Cytoskeleton; Thrombosis.

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