両側腎出血を発症した後天性血友病Aの1例

Abstract

A 82-year-old woman withasymptomatic gross hematuria was referred to us for a consultation. Computed tomography and magnetic resonance imaging revealed hydronephrosis in the right kidney and retrograde pyelography showed a filling defect in the right renal pelvis. Cystoscopy revealed gross hematuria from right ureteral orifice concomitant with superficial papillary bladder tumor (catheterized urine cytology : positive) (transurethral resection of bladder tumor UC G1 pTa). She was diagnosed with right renal pelvic carcinoma, and we performed a total right nephroureterectomy. Histolopathological findings showed no evidence of malignancy, but extensive hemorrhaging was observed in the subepithelium. There was hematoma in the retoroperitoneum and she suffered from endotoxic shock after the operation. In the post-operative period, she showed complications involving left renal bleeding. She was diagnosed with acquired hemophilia A based on a decline in factor VIII activity, prolonged APTT and the presence of anticoagulant. Although we administered steroids to her, her general condition deteriated and she died of postrenal renal failure 37 days after the operation. To our knowledge, this is the fourth case worldwide and the third case in Japan of acquired hemophilia A discovered by renal bleeding.