获得性凝血因子V缺乏症1例并文献复习

Acquired Factor V Deficiency: A Case Report and Literature Review

摘要: 目的：探讨获得性凝血因子V缺乏症(acquired factor V deficiency, AFVD)的临床特点，病因诱因及治疗方法。方法：报告一例AFVD患者的诊治经过，以“AFVD”、“狼疮抗凝物”、“抗磷脂抗体综合征”的中英文为检索词分别在PubMed、CNKI和万方数据知识服务平台检索国内外相关病例，总结AFVD的临床特点、治疗方案及相关诱因。结果：本文报道1例胃癌术后患者凝血功能发生异常，反复发生鼻出血，症状持续1月余并不断加重，医院就诊查血凝常规：凝血酶原时间(PT) 54.90 s，抗凝血酶III (AT-III) 51.40%，活化部分凝血活酶时间(APTT) 144.70 s，PT国际标准化比值(PT-INR) 4.69 INR，PT百分活度(PT%) 21.90%，APTT比值4.67R。进一步检查发现狼疮抗凝物(+)，凝血因子V明显减少(1%)，最终确诊为获得性凝血因子V缺乏症。通过PubMed、CNKI和万方数据知识服务平台检索，共检索到文献206篇，选取2014年到2022年其中9例病例进行临床特点分析，发现出血症状从轻微的粘膜出血到严重的和危及生命的出血均有发生，与凝血因子V水平无明显相关。结论：AFVD是一种罕见的凝血功能异常，通常难以通过单一的病因解释AFVD的发生，治疗上，可以通过血浆置换与免疫抑制治疗使AFVD患者获益。

Abstract: Objective: To investigate the clinical features, etiological factors, and treatment of acquired factor V deficiency (AFVD). Methods: This paper reports the diagnosis and treatment of a patient with AFVD. In addition, the Chinese and English search terms of “AFVD”, “lupus anticoagulant” and “antiphos-pholipid antibody syndrome” were used to search the relevant cases in PubMed, CNKI and Wanfang data knowledge service platform in order to summarize the clinical characteristics, treatment and related causes of AFVD. Results: In this paper, we report a case of a postoperative gastric cancer pa-tient with abnormal coagulation function and recurrent nosebleeds, with symptoms persisting for more than one month and worsening, who presented to the hospital for blood coagulation routine: prothrombin time (PT) 54.90 s, antithrombin III (AT-III) 51.40%, activated partial thromboplastin time (APTT) 144.70 s, PT international normalized ratio (PT-INR) 4.69 INR, PT percent activity (PT%) 21.9%, APTT ratio 4.67R. Further examination revealed lupus anticoagulant (+) and a sig-nificant decrease in coagulation factor V (1%), resulting in a diagnosis of acquired coagulation factor V deficiency. A total of 206 publications were retrieved through PubMed, CNKI, and Wanfang Data-base. Nine of the cases from 2014 to 2022 were selected for clinical characteristics analysis, which revealed that bleeding symptoms ranged from mild mucosal bleeding to severe and life-threatening bleeding, with no significant correlation with coagulation factor V levels. Conclusions: AFVD is a rare coagulation abnormality that is often difficult to explain by a single etiology, and treatment can benefit patients with AFVD through plasma replacement with immunosuppressive therapy.