Familial Thrombocytopenic Thrombocytopathy

Abstract

SUMMARY A unique family with thrombocytopenic thrombocytopathy is described and the relevant literature is reviewed. Two of the more severely affected members who have clinical bleeding and thrombocytopenia were studied in detail. Their platelets are large, fail to adhere to wound surfaces, and are rapidly removed from the circulation. Their platelet thromboplastic activity is sharply reduced relative to their increased content of phospholipid and total lipid. It is postulated that the genetic defect is transmitted as an autosomal dominant.