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成功治疗获得性血友病所致的术后暴发性出血。

Successful treatment of fulminant postoperative bleeding due to acquired haemophilia.

作者信息

Mekenkamp L J M, Beishuizen A, Slomp J, Legdeur M C J C, Klaase J M, Trof R J

机构信息

Departments of Internal Medicine, Medisch Spectrum Twente, Enschede, the Netherlands.

出版信息

Neth J Med. 2015 May;73(4):182-6.

Abstract

Acquired haemophilia is a rare but life-threatening phenomenon in patients who have undergone surgical treatment. We describe a patient with a history of pancreatic cancer and a conventional pancreaticoduodenectomy, who underwent elective resection of an enterocutaneous fistula, complicated by fulminant haemorrhagic shock, caused by acquired haemophilia A. Eventually, the bleeding was controlled by a combination of aggressive haemostatic and immunosuppressive therapy. Prompt diagnosis of acquired haemophilia is crucial to allow early and appropriate haemostatic treatment and reduce the period of increased bleeding risk by eradicating the inhibitor with immunosuppressive therapy.

摘要

获得性血友病在接受手术治疗的患者中是一种罕见但危及生命的现象。我们描述了一名有胰腺癌病史并接受过传统胰十二指肠切除术的患者,该患者接受了肠皮肤瘘的择期切除术,并发由获得性甲型血友病引起的暴发性出血性休克。最终,通过积极的止血和免疫抑制治疗相结合控制了出血。获得性血友病的及时诊断对于尽早进行适当的止血治疗以及通过免疫抑制治疗消除抑制剂来缩短出血风险增加期至关重要。

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