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在一名患有肝细胞癌的特殊患者中发现了针对多种凝血因子(V、IX和XII)的获得性抑制剂。

Acquired Inhibitors to Multiple Coagulation Factors (V, IX, and XII) Identified in a Unique Patient with Hepatocellular Carcinoma.

作者信息

Imataki Osamu, Arai Takeshi, Uemura Makiko

机构信息

Division of Hematology and Stem Cell Transplantation, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Japan.

Department of Laboratory Medicine, Kagawa University Hospital, Japan.

出版信息

Intern Med. 2017;56(10):1203-1206. doi: 10.2169/internalmedicine.56.7845. Epub 2017 May 15.

DOI:10.2169/internalmedicine.56.7845
PMID:28502937
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5491817/
Abstract

An acquired formation of inhibitors to coagulation factors is a rare type of coagulopathy. The development of inhibitors for multiple coagulation factors has never been reported. A 75-year-old Japanese female underwent interventional therapy for hepatocellular carcinoma. Five days after the last intervention, her prothrombin time decreased to 10%, and her activated partial thromboplastin time (APTT) lengthened to 265.1 sec. The activities for coagulation factors showed significantly reduced activities (<10%) of factors V, IX, and XII. A cross-mixing test demonstrated an inhibitor pattern, and inhibitory antibodies against factors V, IX, and XII were detected. We discuss our patient's etiology and pathogenesis.

摘要

获得性凝血因子抑制剂形成是一种罕见的凝血病类型。多种凝血因子抑制剂的出现从未有过报道。一名75岁的日本女性接受了肝细胞癌的介入治疗。最后一次介入治疗五天后,她的凝血酶原时间降至10%,活化部分凝血活酶时间(APTT)延长至265.1秒。凝血因子活性显示因子V、IX和XII的活性显著降低(<10%)。交叉混合试验显示出抑制剂模式,并检测到针对因子V、IX和XII的抑制性抗体。我们讨论了该患者的病因和发病机制。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/00a6/5491817/8be0cbc7dd27/1349-7235-56-1203-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/00a6/5491817/8be0cbc7dd27/1349-7235-56-1203-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/00a6/5491817/8be0cbc7dd27/1349-7235-56-1203-g001.jpg

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本文引用的文献

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Acquired haemophilia: an overview for clinical practice.获得性血友病:临床实践概述。
Eur J Haematol. 2015 Dec;95 Suppl 81:36-44. doi: 10.1111/ejh.12689.
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Transfusion. 2002 Jan;42(1):18-26. doi: 10.1046/j.1537-2995.2002.00011.x.
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Acquired factor V inhibitors.获得性凝血因子V抑制剂
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A survey of 215 non-hemophilic patients with inhibitors to Factor VIII.一项针对215名非血友病性VIII因子抑制物患者的调查。
Thromb Haemost. 1981 Jun 30;45(3):200-3.
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The coagulation cascade: initiation, maintenance, and regulation.凝血级联反应:启动、维持与调节
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