van der Pas Anke J M, Leebeek Frank W G, Perry David J, Castel Ad, van Esser Joost W J
Bronovo Ziekenhuis, Den Haag, The Netherlands.
Ned Tijdschr Geneeskd. 2009;153:B42.
A 69-year-old man of Jewish descent with a second local relapse of rectal carcinoma was found to have a markedly prolonged activated partial thromboplastin time (aPTT). Further evaluation revealed a homozygous factor XI deficiency. Despite various operations in the past he had never displayed any bleeding problems. Severe factor XI deficiency did not prevent venous thrombosis in this patient. The management of patients with prolonged aPTT is described and insight into the pathophysiology of factor XI deficiency is provided. The differential diagnosis in patients with a prolonged aPTT depends on their bleeding tendency. There is a large variability in bleeding tendency in patients with factor XI deficiency. Patients with factor XI deficiency and an increased bleeding tendency can be treated with antifibrinolytic agents prior to small interventions, such as tooth extraction, or with plasma prior to surgery.
一名69岁的犹太裔男性，直肠癌出现第二次局部复发，发现其活化部分凝血活酶时间（aPTT）明显延长。进一步检查发现其存在纯合子因子XI缺乏症。尽管过去进行过多次手术，但他从未出现过任何出血问题。严重的因子XI缺乏症并未阻止该患者发生静脉血栓形成。本文描述了aPTT延长患者的管理方法，并对因子XI缺乏症的病理生理学提供了见解。aPTT延长患者的鉴别诊断取决于其出血倾向。因子XI缺乏症患者的出血倾向差异很大。对于有因子XI缺乏症且出血倾向增加的患者，在进行小手术（如拔牙）前可使用抗纤溶药物治疗，或在手术前使用血浆治疗。
