Takashima Yasushi, Kawanishi Hiroaki, Kotani Shinichi, Watanabe Hiroki, Yokozeki Hitoshi, Funahashi Yurina, Takaoka Naoto, Fujiwara Maki, Okumura Kazuhiro
The Department of Urology, Tenri Hospital.
The Department of Hematology, Tenri Hospital.
Hinyokika Kiyo. 2023 Jun;69(6):169-173. doi: 10.14989/ActaUrolJap_69_6_169.
The patient was a 79-year-old man with ureteroileal anastomotic stricture after a Bricker ileal conduit. Endourological treatment of stenosis was performed via percutaneous nephrostomy and ileal conduit. The patient experienced lower abdominal pain on the following day, and computed tomographic (CT) scan showed hematoma retention around the kidney and active bleeding from the renal artery branches. Transarterial embolisation (TAE) was performed and the bleeding was controlled. Two days later, there was a sudden progression of anemia and CT showed an increase in hematoma around the kidney. We subsequently performed nephrectomy for hemostasis. Five days later, the anemia progressed further. There was hematoma retention in the retroperitoneal cavity, and emergency laparotomy hemostasis was performed. Routine coagulation test results were normal. Heavy bleeding was observed several days after TAE and the possibility of coagulation factor XIII deficiency was considered. Factor XIII deficiency was confirmed by a low factor XIII activity level. The patient was given plasma-derived factor XIII. After receiving factor XIII replacement, factor XIII activity remained unchanged and the patient continued to bleed. Thereafter, a cross-mixing test was performed and the patient was diagnosed with autoimmune acquired factor XIII deficiency. Cortical steroids were administered to remove the factor XIII inhibitor. Steroid administration showed a rapid increase in factor XIII activity, and bleeding symptoms were no longer observed. In cases of serious bleeding of unknown cause with a normal coagulation profile, acquired factor XIII deficiency should be suspected and factor XIII activity measured.
该患者为一名79岁男性，在接受Bricker回肠代膀胱术后出现输尿管回肠吻合口狭窄。通过经皮肾造瘘术和回肠代膀胱术对狭窄进行了腔内治疗。患者在术后第二天出现下腹部疼痛，计算机断层扫描（CT）显示肾脏周围有血肿残留，肾动脉分支有活动性出血。进行了经动脉栓塞术（TAE），出血得到控制。两天后，贫血突然加重，CT显示肾脏周围血肿增加。随后我们进行了肾切除术以止血。五天后，贫血进一步加重。腹膜后腔有血肿残留，遂进行急诊剖腹止血。常规凝血试验结果正常。在TAE术后数天观察到严重出血，考虑存在凝血因子XIII缺乏的可能性。通过低凝血因子XIII活性水平确诊为凝血因子XIII缺乏。给患者输注了血浆源性凝血因子XIII。接受凝血因子XIII替代治疗后，凝血因子XIII活性未改变，患者仍持续出血。此后，进行了交叉配血试验，患者被诊断为自身免疫性获得性凝血因子XIII缺乏。给予皮质类固醇以去除凝血因子XIII抑制剂。使用类固醇后凝血因子XIII活性迅速升高，出血症状不再出现。对于凝血指标正常但原因不明的严重出血病例，应怀疑获得性凝血因子XIII缺乏并检测凝血因子XIII活性。
