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.
D001779:Blood Coagulation Factors / Q000032:analysis; D001780:Blood Coagulation Tests; D005342:Fibrinolysis; D006470:Hemorrhage / Q000097:blood* / Q000235:genetics; D006579:Heterozygote; D006801:Humans; D008297:Male; D008875:Middle Aged; D010976:Platelet Count; D010979:Platelet Function Tests; D012016:Reference Values; D000979:alpha-2-Antiplasmin / Q000172:deficiency* / Q000235:genetics / Q000302:isolation & purification
D001779:Blood Coagulation Factors; D000979:alpha-2-Antiplasmin