Uen W C, Chou Y H, Liu C C, Lin S M, Chen T J
Department of Hematology and Oncology, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
J Formos Med Assoc. 1998 Apr;97(4):283-5.
A 42-year-old-women with sigmoid colon adenocarcinoma was found to have isolated prolonged activated partial thromboplastin time (aPTT 102.5 s, normal range 24-36 s) preoperatively. Her medical history included an episode of prolonged postdelivery uterine bleeding 16 years previously. A mixed aPTT test showed immediate correction of the prolonged aPTT, indicating a coagulation factor deficiency in the intrinsic pathway. Factor assays showed factor XI was below 1% of average normal value whereas factor VIII, IX and XII activities were normal. Family screening revealed one sister among the three siblings also had isolated prolonged aPTT. The patient was transfused with four units (5mL/kg) of fresh frozen plasma the day before surgery, then with two units during surgery. The operation was uneventful with no bleeding problems. The patient recovered smoothly and is currently undergoing adjuvant chemotherapy. This is the first formal report of a patient with factor XI deficiency undergoing major surgery in Taiwan. Careful monitoring of aPTT, with fresh frozen plasma transfusion, when needed, may safely overcome bleeding problems during surgery.
一名42岁患有乙状结肠腺癌的女性在术前被发现活化部分凝血活酶时间(aPTT)延长(aPTT为102.5秒,正常范围为24 - 36秒)。她的病史包括16年前产后子宫出血时间延长。混合aPTT试验显示延长的aPTT立即得到纠正,表明内源性凝血途径存在凝血因子缺乏。因子检测显示因子XI低于平均正常值的1%,而因子VIII、IX和XII活性正常。家族筛查发现三个兄弟姐妹中有一个姐妹也有孤立性aPTT延长。患者在手术前一天输注了4单位(5mL/kg)新鲜冰冻血浆,手术期间又输注了2单位。手术过程顺利,没有出血问题。患者恢复顺利,目前正在接受辅助化疗。这是台湾首例关于因子XI缺乏患者接受大手术的正式报告。仔细监测aPTT,并在需要时输注新鲜冰冻血浆,可能安全地解决手术期间的出血问题。