• To determine the clinical significance of acquired α2-antiplasmin deficiency in patients with bleeding disorders, I reviewed the results of assays performed on 184 patients over a 4-year period. Thirty-two evaluable patients had α2-antiplasmin activity levels of less than 50% of normal (defined as severe deficiency), and 35 patients had levels between 50% and 75% of normal (mild deficiency). Records of these patients and of 32 patients who had normal levels were reviewed. Most patients with severe α2-antiplasmin deficiency had either liver disease or disseminated intravascular coagulation and/or fibrinolysis, or both. There was a high incidence of severe α2-antiplasmin deficiency among patients with acute promyelocytic leukemia. Five patients with pathologic bleeding had no identifiable coagulation abnormalities other than α2-antiplasmin deficiency. The group with severe α2-antiplasmin deficiency had a significantly higher incidence of life-threatening or fatal bleeding and the most striking laboratory evidence of hyperfibrinolysis. Using the presence of severe α2-antiplasmin deficiency as an indication for therapy, ε-aminocaproic acid treatment was associated with cessation of life-threatening bleeding in 8 of 11 patients.
(Arch Intern Med. 1989;149:1769-1772)