结缔组织病相关获得性血友病甲4例报告并文献复习  

Acquired hemophilia A in patients with connective tissue disease: report of 4 cases and review of the literature

作　　者：冯云路[1] 郑文洁[2] 李剑[3] 张文[2] 赵岩[2] 

机构地区：[1]中国医学科学院北京协和医院内科,100730 [2]中国医学科学院北京协和医院风湿免疫科,100730 [3]中国医学科学院北京协和医院血液科,100730

出　　处：《北京医学》2010年第2期83-85,共3页Beijing Medical Journal

摘　　要：目的分析结缔组织病(CTD)相关获得性血友病甲(AHA)的临床特征和治疗方法,加深对该病的认识。方法回顾性分析4例CTD(类风湿关节炎2例,多发性肌炎、未分化结缔组织病各1例)相关AHA患者的临床及实验室检查特点、治疗反应及转归。结果发生AHA时,3例基础病病情活动且未接受过正规治疗,1例多发性肌炎患者经激素和免疫抑制治疗后病情稳定。4例均表现皮肤大片瘀斑和(或)黏膜出血,其中伴关节腔出血1例,消化道出血1例,血尿1例。均存在激活的部分凝血活酶时间(APTT)延长2～4倍,第Ⅷ因子活性(FⅧ∶C)降低(0.31%～5.1%),Ⅷ因子抑制物滴度升高(10～64BU/ml,Bethesda法)。经足量激素联合环磷酰胺或甲氨蝶呤及人免疫球蛋白(IVIG)治疗,并使用新鲜冰冻血浆、VIII因子、凝血酶原复合物(PCC)或活化PCC纠正凝血异常,3例临床出血倾向和APTT明显改善;1例治疗无效。结论AHA可见于多种CTD,病情稳定状态亦可出现。激素联合免疫抑制剂可有效治疗CTD相关的AHA。Objective To investigate the clinical features and therapeutic response of acquired hemophilia A （AHA） in patients with connective tissue disease （CTD） . Methods Retrospective analysis of the clinical course, laboratory result, treatment and outcome of 4 CTD with AHA, including 2 cases of rheumatoid arthritis, one case each of polymyositis（PM） and one undifferentiated CTD case. Results At the onset of AHA, active disease was shown in 3 patients with CTD, while one patient with quiescent PM which was controlled by corticosteroid plus immunosuppressive agents. All patients bad extensive ecchymotic skin lesions and/or mucosa bleeding, accompanyed by hemarthroses in one case, gastrointestinal in one case and urologic bleeding in one case. Laboratory examination revealed 2 to 4 folds prolongation of activated partial thromboplastin time （APTT）, reduced Factor VIII（FVIII ）activity （0.31%-5.1%） and the present of FVIII inhibitor at a titer of 10 to 63 Bethesda units/ml. Transfusion of fresh frozen plasma（FFP） , Human FVIII concentrates, prothrombin complex concentrate （PCC） or activated PCC followed by administration of corticosteroid plus immunosuppressive agents（cyclophos- phamide and/or methotrexate）,and intravenous immunoglobulin successfully arrested bleeding and reduced APTT level in 3 patients. Conclusions Patients with CTD could develop AHA , even in quiescent disease . Corticosteroid combined with immunosuppressive agents are effective in treating CTD associated AHA.

关 键 词：获得性血友病甲 结缔组织病 Ⅷ因子抑制物 治疗 

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