1例系统性红斑狼疮合并获得性血友病A及文献复习  

Systemic lupus erythematosus with acquired hemophilia A:A case report and literature review

作　　者：叶梅 邓锐莹 申凤彩 侯志铎[1,2] 林玲[1,2] YE Mei;DENG Ruiying;SHEN Fengcai;HOU Zhiduo;LIN Ling(Department of Rheumatology and Immunology,First Affiliated Hospital of Shantou University Medical College,Shantou Guangdong 515041;Clinical Research Center,First Affiliated Hospital of Shantou University Medical College,Shantou Guangdong 515041,China)

机构地区：[1]汕头大学医学院第一附属医院风湿免疫科,广东汕头515041 [2]汕头大学医学院第一附属医院临床研究中心,广东汕头515041

出　　处：《中南大学学报（医学版）》2023年第5期789-794,共6页Journal of Central South University ：Medical Science

基　　金：广东省医学科研基金(A2018104);汕头市医疗卫生科技计划(〔2022〕88);汕头大学医学院临床项目提升计划(2014110109)。

摘　　要：系统性红斑狼疮(systemic lupus erythematosus,SLE)合并获得性血友病A(acquired hemophilia A,AHA)罕见,容易延误诊断,且病死率高,有必要进一步加深对该疾病的认识。本文报道1例SLE合并AHA患者的病例特点及治疗方法,并进行文献复习。患者为29岁青年女性,SLE病程10年,以腹腔大出血为主要临床表现,实验室检查活化部分凝血活酶时间(activated partial thromboplastin time,APTT)明显延长(118.20 s),血浆VIII因子活性(coagulation factor VIII activity,FVIII꞉C)极度降低(0.20%),可检测到高滴度抗因子VIII(factor VIII,FVIII)抑制物(31.2 BU/mL)。予大剂量糖皮质激素、大剂量免疫球蛋白、环磷酰胺、利妥昔单抗及输血、补充人凝血因子VIII等治疗后,凝血功能、FVIII꞉C改善,FVIII抑制物(−),未发现严重不良反应。随访5年,病情稳定,无再出血。SLE出现凝血功能障碍,尤其是孤立性APTT延长,需注意排查AHA,糖皮质激素联合免疫抑制剂疗效不佳时,可尝试联合使用利妥昔单抗。Systemic lupus erythematosus(SLE)complicated with acquired hemophilia A(AHA)is a rare condition with frequently delayed diagnosis and a high mortality rate,so it is necessary to strengthen the understanding of this disease.In this study,the characteristics and treatment in 1 case of SLE complicated by AHA is reported and analyzed,and a literature review is conducted.The patient was a 29-year-old young female with a 10-year history of SLE,the main clinical manifestation was severe abdominal bleeding.Laboratory tests revealed that the activated partial thromboplastin time(APTT)was notably prolonged(118.20 s),and the coagulation factor VIII activity(FVIII꞉C)was extremely decreased(0.20%)with high-titer of factor VIII(FVIII)inhibitor(31.2 BU/mL).After treating with high-dose glucocorticoid,immunoglobulin,cyclophosphamide,rituximab,blood transfusion,and intravenous infusion of human coagulation FVIII,the coagulation function and coagulation FVIII꞉C were improved,and FVIII inhibitor was negative without serious adverse reactions.During the next 5-year follow-up,the patient’s condition was stable and no bleeding occurred.In the case of coagulation dysfunction in SLE,especially with isolated APTT prolongation,AHA should be screened.When the therapeutic effects of glucocorticoid combined with immunosuppressants are not desirable,rituximab could be introduced.

关 键 词：系统性红斑狼疮 获得性血友病 利妥昔单抗 

分 类 号：R593.241[医药卫生—内科学] R554.1[医药卫生—临床医学]