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[一名患有因子 XII 缺乏症的患者因输注新鲜冰冻血浆而导致急性肺损伤]

[Acute lung injury as a consequence of fresh frozen plasma administration in a patient with factor XII deficiency].

作者信息

San Juan-Álvarez M, Sánchez-Zamora P, de la Flor-Robledo M

机构信息

Servicio de Anestesiología y Reanimación, Hospital Universitario Severo Ochoa, Leganés, Madrid, España.

Servicio de Anestesiología y Reanimación, Hospital Universitario Severo Ochoa, Leganés, Madrid, España.

出版信息

Rev Esp Anestesiol Reanim. 2014 Oct;61(8):446-50. doi: 10.1016/j.redar.2013.09.012. Epub 2013 Nov 17.

Abstract

Along with the complete blood count, the coagulation tests are those most demanded before a surgical procedure. The activated partial thromboplastin time (APPT) quantifies the intrinsic and common coagulation pathways, including factors XII, XI, IX, VIII, X, V and II. Factor XII deficiency is associated with a prolonged APPT and an increase in thromboembolic phenomena, without increasing the intraoperative bleeding risk. A 20 year old man with factor XII deficiency was receiving two units of fresh frozen plasma because of an APPT of 100 seconds, with the intention of normalizing it before an urgent surgery procedure, and the fear of intraoperative bleeding. An hour after starting the transfusion the patient developed an acute lung injury (ALI) compatible with the diagnosis of a transfusion related acute lung injury (TRALI). The surgery continued without complications, and the patient was admitted to the resuscitation unit for 72 h, needing respiratory support. If the APTT is prolonged in the absence of bleeding, the presence of a non-specific circulating anticoagulant, a deficiency of factor XI, XII and VIII (associated to Von Willebrand disease) must be ruled out. Therefore, in the case presented here, the administration of hemoderivatives was unnecessary and can have consequences as serious as the one that the patient presented, a transfusion related acute lung injury.

摘要

凝血检查与全血细胞计数一样,是外科手术前最常要求进行的检查项目。活化部分凝血活酶时间(APTT)可量化内源性凝血途径和共同凝血途径,包括因子 XII、XI、IX、VIII、X、V 和 II。因子 XII 缺乏与 APTT 延长及血栓栓塞现象增加有关,但不会增加术中出血风险。一名患有因子 XII 缺乏症的 20 岁男性,因 APTT 为 100 秒而接受了两单位新鲜冰冻血浆,目的是在紧急手术前使其恢复正常,并担心术中出血。输血开始一小时后,患者出现了与输血相关急性肺损伤(TRALI)诊断相符的急性肺损伤(ALI)。手术继续进行且无并发症,患者被收入复苏病房 72 小时,需要呼吸支持。如果在没有出血的情况下 APTT 延长,必须排除存在非特异性循环抗凝剂、因子 XI、XII 和 VIII 缺乏(与血管性血友病相关)的情况。因此,在此病例中,给予血液制品是不必要的,并且可能会产生与患者所出现的情况一样严重的后果,即输血相关急性肺损伤。

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