CAJ | 학술논문

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.
목적 분석결체조직병(CTD)상관획득성혈우병갑(AHA)적림상특정화치료방법,가심대해병적인식.방법 회고성분석4례CTD(류풍습관절염2례,다발성기염、미분화결체조직병각1례)상관AHA환자적림상급실험실검사특점、치료반응급전귀.결과 발생AHA시,3례기출병병정활동차미접수과정규치료,1례다발성기염환자경격소화면역억제치료후병정은정.4례균표현피부대편어반화(혹)점막출혈,기중반관절강출혈1례,소화도출혈1례,혈뇨1례.균존재격활적부분응혈활매시간(APTT)연장2～4배,제Ⅷ인자활성(FⅧ:C)강저(0.31%～5.1%),Ⅷ인자억제물적도승고(10～64BU/ml,Bethesda법).경족량격소연합배린선알혹갑안접령급인면역구단백(IVIG)치료,병사용신선빙동혈장、Ⅷ인자、응혈매원복합물(PCC)혹활화PCC규정응혈이상,3례림상출혈경향화APTT명현개선;1례치료무효.결론 AHA가견우다충CTD,병정은정상태역가출현.격소연합면역억제제가유효치료CTD상관적AHA.
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 had 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(cyclophosphamide 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.