Plasma alpha 2-antiplasmin activity. Role in the evaluation and management of fibrinolytic states and other bleeding disorders.
To determine the clinical significance of acquired alpha 2-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 alpha 2-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 alpha 2-antiplasmin deficiency had either liver disease or disseminated intravascular coagulation and/or fibrinolysis, or both. There was a high incidence of severe alpha 2-antiplasmin deficiency among patients with acute promyelocytic leukemia. Five patients with pathologic bleeding had no identifiable coagulation abnormalities other than alpha 2-antiplasmin deficiency. The group with severe alpha 2-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 alpha 2-antiplasmin deficiency as an indication for therapy, epsilon-aminocaproic acid treatment was associated with cessation of life-threatening bleeding in 8 of 11 patients.
Williams
E C
EC
Department of Medicine, University of Wisconsin, Madison.
D000293:Adolescent; D015119:Aminocaproic Acid / Q000627:therapeutic use; D001778:Blood Coagulation Disorders / Q000097:blood* / Q000188:drug therapy; D002648:Child; D004211:Disseminated Intravascular Coagulation / Q000097:blood / Q000188:drug therapy; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005340:Fibrinogen / Q000032:analysis; D005342:Fibrinolysis; D006801:Humans; D015473:Leukemia, Promyelocytic, Acute / Q000097:blood / Q000188:drug therapy; D008107:Liver Diseases / Q000097:blood; D008297:Male; D009362:Neoplasm Metastasis; D000979:alpha-2-Antiplasmin / Q000172:deficiency / Q000502:physiology*
D005338:Fibrin Fibrinogen Degradation Products; D000979:alpha-2-Antiplasmin; D005340:Fibrinogen; D015119:Aminocaproic Acid