Severe hemorrhagic tendency in heterozygous alpha 2-antiplasmin deficiency

We report a case of partial deficiency of alpha 2-antiplasmin. A 45 year old Spanish man had a life long severe bleeding tendency after trauma. Routine coagulation and platelet functional tests in symptomatic and asymptomatic periods were normal. During the bleeding there was a rapid whole blood clot lysis and the concentration of alpha 2-antiplasmin in plasma was 20 to 25% of normal controls. After recovery these levels showed a slight increase (35 to 45% of normal). Addition of normal plasma to the patient's plasma increased alpha 2-plasmin inhibitor activity. Bidimensional electrophoresis of alpha 2-antiplasmin in the patient's plasma showed a normal pattern. Family studies showed that one of the proband's two sons had a mild hemorrhagic tendency and 40% of functional and antigenic levels of alpha 2-antiplasmin.