C1-ESTERASE INHIBITOR TRANSFUSIONS IN PATIENTS WITH HEREDITARY ANGIOEDEMA

Background: Hereditary angioedema results from the deficiency of C1-es terase inhibitor (C1-INH), and C1-INH replacement would represent defi nitive treatment for angioedema attacks. In Canada, C1-INH is availabl e only on a compassionate basis at select medical facilities. Our obje ctive is to assess the efficacy of C1-WH transfusions during angioedem a attacks at a single Canadian institution. Methods: A retrospective c hart review of transfusion data between January 1, 1995 and June 30, 1 996 was performed. Phone interviews with patients elicited their opini ons of the treatment. Data collected included the number and duration of angioedema attacks, dose of transfused C1-INH, and side effects of treatment. Results: Of a cohort of 13 patients with hereditary angioed ema, seven received transfusions with C1-INH. Attacks totaled 87, and more than 100,000 units of the product were transfused. The mean time for abatement of an attack after initiation of transfusion was 50 +/- 8 minutes (1 SD). Then were no reports of adverse effects. Although pa tients were satisfied with the treatment, they raised concerns regardi ng long-term safety and availability. Conclusions: C1-LNH transfusion is a satisfactory means of treating angioedema attacks.