A 63-year-old woman, without a family history of
hemophilia, was admitted to our hospital because of subcutaneous
bleeding, intramuscular and intra-articular
hematoma, and macroscopic
hematuria. On routine blood analysis, a prolonged activated partial thromboplastin time, decreased concentration of
factor VIII to less than 1%, and a markedly elevated level of
factor VIII inhibitor to 14.1 BU/ml were revealed. Diagnosis of acquired
hemophilia was made and she was treated with
prednisolone and recombinant
activated factor VII (
rFVIIa). On day 9 of
rFVIIa therapy, she was complicated by
acute renal failure (ARF) with increasing macroscopic
hematuria. Computed tomography revealed bilateral swelling of the kidneys with
bleeding and dilatation of the left renal pelvis. Activated
prothrombin complex concentrates (aPCC) was administrated in combination with
steroid pulse
therapy and hydration. The
bleeding tendency, including ARF, was improved with aPCC, and she was treated with
prednisolone and
cyclophosphamide. She is currently in good health and attending an outpatients' clinic. Acquired
hemophilia is associated with various underlying conditions, but our patient did not show any previous history. ARF is a rare complication in acquired
hemophilia, requiring a non-invasive treatment combination with early induction of immunosuppressive therapy.