单中心7例获得性血友病A的回顾性临床研究  

A single-center retrospective clinical study of 7 cases of acquired hemophilia A

作　　者：钱玲玲 裴孝平[1] 孙爱红[1] 何斌[1] 孙梅[1] QIAN Lingling;PEI Xiaoping;SUN Aihong;HEI Bin;SUN Mei(Northern Jiangsu People’s Hospital,Jiangsu 225001,China)

机构地区：[1]江苏省苏北人民医院血液科,江苏225001

出　　处：《诊断学理论与实践》2024年第5期524-530,共7页Journal of Diagnostics Concepts & Practice

摘　　要：目的:探讨获得性血友病A(Acquired hemophilia A,AHA)的病因、临床表现、诊治及预后,提高临床对AHA的认识及诊治水平。方法:对我院2013年1月至2023年12月诊断的7例AHA患者的进行回顾性分析,并分别以“Acquired hemophilia A”及“获得性血友病A”在PubMed及中国知网、万方数据库中检索相关文献,从一般资料、病因、诊断时间、临床表现、治疗及预后方面进行对比分析。结果:7例AHA患者均为女性,中位年龄为44.1岁(26~68岁),1例继发于乙型病毒性肝炎感染,1例继发于妊娠,1例为消化道手术后发病,1例继发于干燥综合征。皮肤瘀斑是AHA患者最主要临床表现(7/7),且均有活化部分凝血活酶时间(activated partial thromboplastin time,APTT)升高、凝血因子Ⅷ(factorⅧ,FⅧ)活性(FⅧ:C)降低、FⅧ抗体(FⅧ:Ab)升高。有6例患者接受止血治疗,所有患者接受清除抗体治疗。本组中位随访391 d(28~777 d),除1例患者失访,4例患者达到完全缓解(complete response,CR),达到CR的中位时间为40 d(27~48 d),1例患者达到部分缓解(partial response,PR),均未出现复发。1例患者治疗无效(no response,NR),在随访期间无新发临床出血表现。文献检索到1252例,AHA男女均可发病,男女比例为1.18:1,男女中位年龄分别为65.6岁、55.8岁。56.8%AHA患者为特发型,11.4%与肿瘤、11.1%与自身免疫性疾病、6.7%与妊娠等相关。55%患者接受旁路途径药物是止血治疗,清除抗体治疗多采用以糖皮质激素、环磷酰胺及利妥昔单抗为基础的方案。结论:AHA可继发于肿瘤、自身免疫性疾病、感染、妊娠等,患者多有皮肤淤斑;旁路途径止血、免疫抑制治疗清除抗体,多数患者预后良好。Objective This study aims to explore the etiology,clinical manifestations,diagnosis and treatment,and prognosis of acquired hemophilia A(AHA),and to improve the clinical understanding,diagnosis,and treatment of AHA.Methods A retrospective analysis was conducted on 7 cases of AHA diagnosed at our hospital from January 2013 to December 2023.Relevant literature was searched in PubMed,CNKI,and Wanfang databases using the terms “Acquired hemophilia A”.A comparative analysis was performed on general data,etiology,diagnosis time,clinical manifestations,treatment,and prognosis.Results All 7 AHA patients were female,with a median age of 44.1 years(range 26-68 years).One case was secondary to hepatitis B virus infection;one was secondary to pregnancy;one developed after gastrointestinal surgery;and one was secondary to Sj?gren's syndrome.Skin ecchymosis was the most common clinical manifestation of AHA(7/7),with increased activated partial thromboplastin time(APTT),decreased coagulation factor Ⅷ(FⅧ) activity(FⅧ:C),and increased FⅧ antibody(FⅧ:Ab).Six patients received hemostatic therapy,and all patients received antibody clearance therapy.The median follow-up time was 391 days(28-777 days),with one patient lost to follow-up.Four patients achieved complete response(CR),with a median time to CR of 40 days(27-48 days).One patient achieved partial response(PR),and no recurrence occurred.One patient had no response(NR) and no new clinical bleeding during the follow-up period.The literature review found that AHA can occur in both men and women,with a predominance of women of childbearing age.56.8% of the patients had idiopathic AHA,while 11.4%,11.1%,and 6.7% patients of AHA associated with tumors,autoimmune diseases,and pregnancy,respectively.The male to female ratio is 1.18:1,with median ages of 65.6 years and 55.8 years,respectively.Bypass pathway drugs were the first choice for hemostatic treatment,and antibody clearance therapy was mostly based on glucocorticoids,cyclophosphamide,and rituximab.Conclusions AHA c

关 键 词：获得性血友病A 诊断 治疗 

分 类 号：R446.1[医药卫生—诊断学]