A patient with systemic lupus erythematosus (SLE) developed acquired hemoph
ilia A. The patient, a 24-year-old Japanese woman, was referred to our hosp
ital because of uncontrollable bleeding following a tooth extraction. Labor
atory examination revealed prolonged APTT (116 seconds), reduced factor VII
I activity (2.8 %) and the presence of factor VIII inhibitor at a titer of
46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and
activated prothrombin complex concentrate followed by administration of pre
dnisolone and cyclophosphamide successfully arrested bleeding and reduced t
he factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal
condition. Rapid diagnosis and introduction of adequate therapies are criti
cal.