DIAGNOSIS AND TREATMENT OF AN ACQUIRED FA CTOR-VIII INHIBITOR IN COMBINATION WITH LUPUS ANTICOAGULANTS

History and clinical findings: A 63-year-old woman, known to have a pr imary factor VIII inhibitor (FFI) in combination with lupus anticoagul ants (LA) was hospitalised because of life-threatening bleeding from m outh and neck. Investigations: The activity of coagulation factor VIII was 9% under substitution, while the factor VIII inhibitor titre was 123 U/ml. A lupus anticoagulant test was positive. Antibodies against varicella-zoster virus and Epstein-Barr virus were demonstrated. The r ight adrenal was found to be enlarged on computed tomography. Treatmen t and course: Coagulation became normal on administration of porcine f actor VIII concentrate. Three cycles of a combination of three protein A immunoadsorptions, cyclophosphamide (twice 1.0 g intravenously), Ig G (30 g daily for 5 days) as well as long-term oral cyclophosphamide a dministration (150 mg daily) during the interval were undertaken to re duce the inhibitor and produce immune-tolerance. The factor VIII inhib itor titre was stabilised at a low level, but factor VIII activity cou ld not be normalised without substitution. Conclusions: The simultaneo us presence of specific and nonspecific inhibitors makes laboratory di agnosis and treatment more difficult. Porcine factor VIII and a combin ation of immunoadsorption and suppression are important components in the treatment of bleeding episodes and the production of immunotoleran ce.