乳腺癌术后获得性血友病A的临床研究  

A case of acquired haemophilia A after radical mastectomy

作　　者：顾怡[1] 周景艺 沈薇[1] GU Yi;ZHOU Jingyi;SHEN Wei(Department of Laboratory Medicine,Renji Hospital,Shanghai Jiaotong University School of Medicine,Shanghai 200127,China)

机构地区：[1]上海交通大学医学院附属仁济医院检验科,上海200127

出　　处：《检验医学与临床》2024年第16期2445-2448,共4页Laboratory Medicine and Clinic

基　　金：国家自然科学基金:资助项目(82272397)。

摘　　要：目的 探讨1例乳腺癌术后获得性血友病A(AHA)的病因、临床表现、诊断与治疗过程,并通过检索文献,从实验室检查的角度出发,总结AHA的成因、诊断、治疗、预防等情况,以提高临床对AHA的认识。方法 分析该院2023年5月13日收治的1例乳腺癌术后患者创面渗血不止的原因,同时进行活化部分凝血活酶时间(APTT)纠正试验和凝血因子Ⅷ抑制物检测。以“获得性血友病A”“AHA”为关键词,对万方数据库和PubMed数据库2015-2024年发表的中文文献进行检索并分析。结果 该例患者APTT孤立性延长伴凝血因子Ⅷ∶C显著降低。APTT纠正试验提示患者存在时间温度依赖性凝血因子Ⅷ抗体,凝血因子Ⅷ抑制物滴度为1∶16.。该患者临床诊断为AHA,经注射重组活化人凝血因子Ⅶ止血,加用环磷酰胺清除凝血因子Ⅷ抑制物,患者血红蛋白水平逐渐稳定升高。文献检索结果发现,共检索到文献15篇,其中单病例报道的有10篇,多病例报道的有5篇,共涉及78个病例,其中无明确病因35例,自身免疫性疾病19例,约50%病例伴有基础疾病。结论 外科手术一定要重视凝血功能检测,对于孤立性APTT延长的患者,最好查找APTT延长的根本原因,同时临床要提高对AHA的识别、诊断和治疗水平。Objective To explore the etiology,clinical manifestations,diagnosis and treatment process of a case of acquired hemophilia A(AHA)after breast cancer surgery,and to summarize the causes,diagnosis,treatment and prevention of AHA from the view of laboratory examination by searching the literature,in order to improve the clinical understanding of AHA.Methods One breast cancer patients with postoperative wound bleeding enrolled in the hospital on May 13,2023 was analyzed,and the activated partial thromboplastin time(APTT)correction test and coagulation factorⅧinhibitor test were performed at the same time.The Chinese literatures published in the Wanfang database and PubMed database from 2015 to 2024 were searched by the keywords"acquired hemophilia A"and"AHA"and analyzed.Results Isolated prolongation of APTT was accompanied by a significant decrease in coagulation factorⅧ∶C in this patient.The APTT correction test suggested the presence of time-temperature-dependent coagulation factorⅧantibodies in this patient,and the titer of coagulation factorⅧinhibitor was 1∶16.The patient was clinically diagnosed as AHA,whose hemoglobin level gradually increased after injection of recombinant activated human coagulation factorⅫto stop bleeding and the addition of cyclophosphamide to remove the coagulation factorⅧinhibitor.Literature search results revealed that 15 articles were retrieved,including 10 single case reports and 5 multiple cases reports,involving a total of 78 cases,of which 35 cases without clear etiology,19 cases with autoimmune diseases,and about 50%of the cases were accompanied with primary diseases.Conclusion Coagulation function test should be paid more attentions in surgery,especially for patients with isolated APTT prolongation,it is better to find the primary cause of APTT prolongation,meanwhile the clinic should improve the levels of recognition,diagnosis and treatment of AHA.

关 键 词：获得性血友病A 乳腺癌 重组活化人凝血因子Ⅶ 活化部分凝血酶时间 凝血因子Ⅷ抑制物 

分 类 号：R737.9[医药卫生—肿瘤] R554.1[医药卫生—临床医学]