结缔组织病合并获得性血友病甲八例临床分析  

Clinical analysis of 8 cases of connective tissue disease complicated with acquired hemophilia A

作　　者：杨艳丽[1] 乔鹏燕[1] 陈昱 许珂[1] 张改连[1] 张莉芸[1] Yang Yanli;Qiao Pengyan;Chen Yu;Xu Ke;Zhang Gailian;Zhang Liyun(Department of Rheumatology and Chinical Immunology,Shanxi Dayi Hospital,Shanxi Academy of Medical Sciences,Taiyuan 030032,China)

机构地区：[1]山西医学科学院,山西大医院风湿免疫科,太原030032

出　　处：《中华风湿病学杂志》2019年第4期259-262,共4页Chinese Journal of Rheumatology

摘　　要：目的分析CTD并发获得性血友病甲(AHA)的临床特点及治疗方案。方法回顾性分析8例CTD(SLE5例,SS2例,RA1例)相关AHA患者的临床表现、诊断方法、治疗方案及转归。结果8例CTD患者发生AHA时,7例基础病病情活动,5例出现不同部位的出血症状。5例SLE中并发抗磷脂综合征者3例,其中2例有血栓形成。8例患者均存在激活的部分凝血活酶时间(APTT)延长1.7~3.1倍,凝血因子Ⅷ活性(FⅧ:C)为9.2%~21%(50%~150%),而Ⅷ因子抑制物滴度升高达7.6~56BU/ml(Bethesda法)。7例给予足量激素、免疫抑制剂、人免疫球蛋白(IVIG)、血液制品等方法治疗,5例患者临床出血倾向和APTT明显改善,1例治疗无效。结论CTD易合并AHA,糖皮质激素联合免疫抑制剂可有效治疗CTD相关的AHA,对于难治性患者可酌情选择利妥昔单抗治疗。To analyze the clinical features and treatment of connective tissue disease (CTD) complicated with acquired hemophilia A (AHA).Methods A retrospective analysis of 8 cases of CTD [5 cases of systemic lupus erythematosus (SLE),2 cases of Sjogren's syndrome (SS),1 case of rheumatoid arthritis (RA)] related to clinical manifestations,diagnostic methods,treatment options and outcomes.Results At the onset of AHA,active disease was shown in 7 patients with CTD,and 5 cases had bleeding symptoms in different parts.There were 3 cases of anti-phospholipid syndrome in 5 cases of SLE,2 of which had thrombosis.In 8 patients,the activated partial thromboplastin time (APTT) was prolonged by 1.7 to 3.times,FⅧ: C was 9.2% to 21%(50% to 150%),and the factor Ⅷ inhibitor titer was increased by 7.6 to 56 BU/m1 (Bethesda method).Seven patients were treated with sufficient hormones,immunosuppressive agents,human immunoglobulin (IVIG),and blood products.Five patients had clinically improved bleeding tendency and APTT,and one patient was ineffective.Conclusion CTD is easy to combine with AHA.Glucocorticoid combined with immunosuppressive agent can effectively treat CTD-related AHA.For refractory patients,rituximab can be an alternative.

关 键 词：结缔组织疾病 红斑狼疮 系统性 干燥综合征 关节炎 类风湿 获得性血友病甲 部分凝血活酶时间延长 利妥昔单抗 

分 类 号：R593.2[医药卫生—内科学] R554.1[医药卫生—临床医学]