Platelet Survival Studies in Aldrich Syndrome

Abstract

1. Normal platelets were transfused into 4 patients with Aldrich syndrome. The response in platelet count indicated that little destruction of platelets was occurring. The survival of platelets was considered to be normal. Plasma transfusion alone did not correct the platelet counts. Platelet antibodies were not detected in two of these patients. 2. For control studies, normal platelets were transfused into two patients with amegakaryocytotic congenital thrombocytopenia and sex-linked recessive (non-Aldrich) familial thrombocytopenia. Their survival was normal. Similarly, normal platelets were transfused into three patients with idiopathic thrombocytopenic purpura. Minimal increase in platelets and no significant length of survival of platelets was seen. The thrombocytopenia of Aldrich syndrome was considered to be due to a defect in procduction and/or release of platelets.