Acquired Factor VIII Inhibitor and Cardiopulmonary Bypass

Abstract

ACQUIRED HEMOPHILIA A (AHA) may cause massive bleeding during surgical procedures due to the formation of specific autoantibodies most frequently against coagulation factor VIII (FVIII), which decrease its activity and plasma half-life. Whereas up to 30% of chronically substituted patients with inherited hemophilia A develop inhibitor activity during their treatment, the incidence of AHA is extremely low at 1.5 cases per million. 1 Rodriguez-Merchan E.C. Rocino A. Literature review of surgery management in inhibitor patients. Haemophilia. 2004; 10: 22-29 Crossref PubMed Scopus (35) Google Scholar , 2 Collins P.W. Hirsch S. Baglin T.P. et al. Acquired hemophilia A in the United Kingdom: A 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood. 2007; 109: 1870-1877 Crossref PubMed Scopus (526) Google Scholar Elderly people (aged 60-80 years) are affected especially, although there is another peak at a younger age (20-30 years). 3 Green D. Lechner K. A survey of 215 non-hemophilic patients with inhibitors to factor VIII. Thromb Haemost. 1981; 45: 200-203 Crossref PubMed Scopus (681) Google Scholar AHA is associated with malignancy (11.8%), autoimmune diseases (13.4%), pregnancy (8.4%), drugs (3.4%), and dermatologic diseases (1.4%). In the majority of patients, the cause remains unknown. 4 Knoebl P. Marco P. Baudo F. et al. Demographic and clinical data in acquired hemophilia A: Results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012; 10: 622-631 Crossref PubMed Scopus (316) Google Scholar