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获得性血友病A作为反复胸腔出血的病因——病例报告及文献综述

Acquired hemophilia A as a cause of recurrent bleeding into the pleural cavity - case report and literature review.

作者信息

Wojtyś Małgorzata, Żuk Ewa, Alchimowicz Jacek, Grodzki Tomasz

机构信息

Department of Thoracic Surgery and Transplantation, Pomeranian Medical University in Szczecin, Poland.

Department of Internal Medicine, Health Care Centre "Zdroje" in Szczecin, Poland.

出版信息

Kardiochir Torakochirurgia Pol. 2014 Sep;11(3):329-35. doi: 10.5114/kitp.2014.45686. Epub 2014 Sep 28.

Abstract

Acquired hemophilia A is a coagulation disorder caused by autoantibodies against blood coagulation factor VIII. The first sign of this disease is often massive bleeding, which can affect patients after routine procedures. The parameter which indicates the presence of this condition is isolated prolonged activated partial thromboplastin time (APTT). The present article describes a case of a 32-year-old man with acute interstitial pneumonia and pleural effusion, in whom a massive hemothorax appeared after thoracocentesis; active bleeding was observed after the introduction of a chest tube. The patient was operated on, and no pinpoint bleeding was discovered during the procedure. Active bleeding was still taking place postoperatively. The patient underwent another operation after 6 days. Once more, no pinpoint bleeding was found. Prolonged APTT was observed. The activity of blood coagulation factor VIII was 3.04%. The presence of antibodies against factor VIII was confirmed, and acquired hemophilia was diagnosed. The article also includes an analysis of the literature on acquired hemophilia.

摘要

获得性血友病A是一种由针对凝血因子VIII的自身抗体引起的凝血障碍。这种疾病的首个迹象通常是大量出血,在常规手术后可能会影响患者。表明存在这种情况的参数是孤立的活化部分凝血活酶时间(APTT)延长。本文描述了一名32岁男性急性间质性肺炎并伴有胸腔积液的病例,该患者在胸腔穿刺后出现大量血胸;在置入胸管后观察到活动性出血。患者接受了手术,术中未发现点状出血。术后仍有活动性出血。患者在6天后再次接受手术。再次未发现点状出血。观察到APTT延长。凝血因子VIII的活性为3.04%。确认存在针对因子VIII的抗体,诊断为获得性血友病。本文还包括对获得性血友病相关文献的分析。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f771/4283895/56f09bbcbc02/KITP-11-23601-g001.jpg

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