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Acquired haemophilia A presenting as acute renal failure secondary to bilateral renal bleeding

A 71-year-old man was referred to our hospital with asymptomatic gross haematuria. His medical history showed lung tuberculosis when in his twenties. There was no history of bleeding tendency and no present medication; no familial history of haemophilia was reported. A physical examination and laboratory tests showed no abnormal findings, and his thrombocyte count was normal. Cystoscopy showed bleeding from the bilateral ureteric orifice, and flexible ureteroscopy showed a diffuse reddish lesion of the bilateral pelvic mucosae. Ten days after the initial examination he was immediately admitted after complaining of loin pain and severe haematuria, and presented with postrenal acute renal failure (ARF) from numerous clots in the bilateral upper urinary tracts. His blood urea nitrogen and serum creatinine were elevated to 508 and 65 mg/L, respectively. Blood coagulatory function tests showed a prolonged activated partial thromboplastin time (APTT) of 85.2 s and a normal prothrombin time (PT) of 10.5 s. Factor assays then confirmed an isolated low factor VIII coagulant activity of < 1%, with evidence of factor VIII inhibitor of 59 Bethesda units/mL. Consequently, the patient was diagnosed as having acquired haemophilia A (AHA). Initially he was given factor VIII for 3 days and 60 mg of prednisolone daily for 2 weeks. However, his coagulatory function did not improve, prompting oral administration of 100 mg of cyclosporin twice daily. Two months later, his renal function, APTT and factor VIII activity improved, and the level of factor VIII inhibitor had fallen to undetectable levels. At the 6-month follow-up he received cyclosporin therapy with no recurrence of haematuria or any adverse reaction. Moreover, his coagulatory function remains normal. The changes in laboratory data over the treatment course are summarized in Table 1.