血液透析患者合并获得性凝血因子Ⅴ缺乏症  

Acquired factor Ⅴ deficiency in a hemodialysis patient

作　　者：高佩娟 黄文辉[1] 魏小芳 李莹屏[1] 黄军悦[1] 李小丽[1] 赵慧[1] 张健 董慧 薛嵘[1] GAO Peijuan;HUANG Wenhui;WEI Xiaofang;LI Yingping;HUANG Junyue;LI Xiaoli;ZHAO Hui;ZHANGJian;DONG Hui;XUE Rong(Division of Nephrology,Gansu Provincial Hospital,Lanzhou 730000,China;Division of Hematology,Gansu Provincial Hospital,Lanzhou 730000,China)

机构地区：[1]甘肃省人民医院肾内科,兰州730000 [2]甘肃省人民医院血液科,兰州730000

出　　处：《肾脏病与透析肾移植杂志》2023年第1期95-99,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：兰州市级课题科技计划项目(2020-ZD-23);甘肃省基础研究计划-自然科学基金(22JR11RA256)。

摘　　要：42岁男性患者,规律血液透析1月,因“乏力、气短1 d”就诊。实验室检查显示凝血酶原时间、活化部分凝血活酶时间(APTT)显著延长,APTT纠正试验无法纠正,凝血酶时间正常,凝血因子Ⅴ活性降低,抑制物检测阳性,予以糖皮质激素单药治疗后凝血功能恢复正常。患者预后良好,在一年的随访中未复发。获得性凝血因子Ⅴ缺乏症很罕见,临床表现具有异质性,在有基础病患者群中预后不良,及时的诊断和治疗有助于改善患者预后,但需警惕药物相关并发症。A 42-year-old male on regular hemodialysis for one month was referred to our hospital because of weakness and shortness of breath for one day. Laboratory tests showed markedly prolonged prothrombin time( PT) and activated partial thromboplastin time( APTT),which failed to be corrected by APTT mixing test,and thrombin time( TT)was normal. Factor Ⅴ( FV) activity was decreased and the FV inhibitor was detected. Blood coagulation function returned to normal after glucocorticoid therapy. The patient had a good prognosis and was recurrence-free during one year of followup. Acquired factor Ⅴ deficiency is a rare coagulopathy with a variety of clinical presentations,and has a poor prognosis in patients with chronic disease. Early diagnosis and treatment can improve the prognosis,and clinicians need to be aware of drug-associated complications.

关 键 词：尿毒症 血液透析 获得性凝血因子Ⅴ缺乏症 获得性凝血因子Ⅴ抑制物 糖皮质激素 

分 类 号：R692.5[医药卫生—泌尿科学]