Gurcan Mehmet, Turan Serdar, Demirel Ebru, Tonyali Senol
Urology, Istanbul University School of Medicine, Istanbul, TUR.
Anesthesiology, Istanbul University School of Medicine, Istanbul, TUR.
Cureus. 2023 Mar 16;15(3):e36255. doi: 10.7759/cureus.36255. eCollection 2023 Mar.
Factor XI deficiency (hemophilia C or Rosenthal syndrome) is an inherited rare disorder that leads to abnormal bleeding due to the paucity of the protein named factor XI, which plays a role in the blood clotting cascade. A 42-year-old male was referred to the urology outpatient clinic with macroscopic hematuria. The patient was scheduled for a repeat transurethral resection of a bladder tumor (TURBT). Preoperative coagulation parameters were as follows: the international normalized ratio (INR) was 0.95 (0.85-1.2), the prothrombin time was 10.9 seconds (10-15), and the partial thromboplastin time was 43.7 seconds (21-36). On the second postoperative day, he developed pelvic pain and discomfort. An abdominal CT revealed a 10 cm mass consistent with clot retention. The patient received two units of erythrocyte suspension and six units of fresh frozen plasma to prevent the depletion of hemoglobin and control urinary bleeding. The patient was discharged with a good recovery from the hospital three days after the second surgery. Hematologic disorders are rare but might have fatal consequences following surgery if unnoticed at the earliest stage. Clinicians must consider that patients with a history of unusual bleeding or borderline coagulation parameters might have an underlying hematological disorder and perform a further evaluation.
因子 XI 缺乏症(血友病 C 或罗森塔尔综合征)是一种遗传性罕见疾病,由于名为因子 XI 的蛋白质缺乏,导致异常出血,该蛋白质在血液凝固级联反应中起作用。一名 42 岁男性因肉眼血尿被转诊至泌尿外科门诊。该患者计划再次行经尿道膀胱肿瘤切除术(TURBT)。术前凝血参数如下:国际标准化比值(INR)为 0.95(0.85 - 1.2),凝血酶原时间为 10.9 秒(10 - 15),活化部分凝血活酶时间为 43.7 秒(21 - 36)。术后第二天,他出现盆腔疼痛和不适。腹部 CT 显示一个 10 厘米的肿块,符合血块潴留。患者接受了两单位红细胞悬液和六单位新鲜冰冻血浆,以防止血红蛋白耗竭并控制血尿。第二次手术后三天,患者康复出院。血液系统疾病很少见,但如果在最早阶段未被发现,手术后可能会产生致命后果。临床医生必须考虑到有异常出血史或凝血参数临界的患者可能存在潜在的血液系统疾病,并进行进一步评估。