Wiskott—Aldrich Syndrome: Qualitative Platelet Defects and Short Platelet Survival

摘要: Summary. Investigations of the haemostatic functions in three patients with Wiskott-Aldrich syndrome are presented. All had severe thrombocytopenia and prolonged bleeding times. The platelets abnormal morphology reduced size variations shape. Electron microscopy revealed ultrastructural abnormalities a number organelles, many contained large numbers tubules. Platelet electrophoretic mobility citrated plasma was not by collagen, platelet aggregation collagen ADP deficient. Biochemical studies lack storage pool adenine nucleotides. adhesiveness vitro whole blood reduced. factor-3 release kaolin, freezing thawing normal one another patients. Platelet survival showed time donor patient, while autologous markedly two patients. bone marrow megakaryocytes. By electron marrow, were found to be phagocytosed macrophages reticulum cells. main cause is most probably incrcased peripheral destruction platelets. It suggested that qualitatively defective recognized reticulo-endothelial system as foreign particles phagocytosed.