先天性第Ⅴ因子欠乏症に合併した尿管腫瘍

A PATIENT OF URETERAL TUMOR WITH CONGENITAL FACTOR V DEFICIENCY

　68歳，男性．無症候性血尿を主訴に来院．経皮的腎孟造影，CTにて右尿管腫瘍を疑い，右腎尿管全摘除術を予定．しかし，術前に，先天性第Ⅴ因子欠乏症（パラ血友病）と診断され，新鮮凍結血漿大量輸血により第Ⅴ因子を補い，プロトロンビン時間，活性化部分トロンボプラスチン時間を補正させた後に右腎尿管全摘除術を施行した．術後も新鮮凍結血漿を輸血し，経過は良好であった．病理組織学的診断は，右尿管腫瘍（移行上皮癌，grade Ⅲ）であった．

A 68-year-old man visited our hospital because of asymptomatic macroscopic hematuria. Percutaneous pyelography and CT scan revealed right ureteral tumor. We found the patient had congenital factor V deficiency (parahemophilia), therefore a large amount of fresh frozen plasma was transfused to improve prothrombin time and activated partial thromboplastin time. We performed right nephroureterectomy successfully and transfused fresh frozen plasma postoperatively. There was no serious tendency of hemor-rhage during the operation. There was no serious trouble except for slight elevation of BUN level, which improved by low-protein diet.