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目的巨噬细胞活化综合征(MAS)的诊断和治疗尚未得到统一,总结分析我院6例MAS患儿的临床资料,为临床救治工作提供参考。方法回顾性分析我院1998—2004年风湿性疾病合并MAS患儿的临床资料。结果共有6例(女4例,男2例)患儿符合MAS的诊断条件。发生MAS前,4例诊断幼年特发性关节炎全身型(SOJIA),2例诊断系统性红斑狼疮和狼疮性肾炎。所有病例确诊MAS时疾病状态处于低活动期,主要的临床特征是:峰形热(5/6)或中高热(1/6),肝脾肿大(6/6),淋巴结病(6/6),外周血3系减少(6/6),明显的肝损伤(6/6),弥漫性血管内凝血象(2/6)和中枢神经系统功能障碍(3/6)。低纤维蛋白原血症、肝酶升高和高甘油三酯血症均持续存在。3例骨髓涂片可见吞噬血细胞现象和浆细胞岛,其他3例未行此检查。3例患儿被证实存在感染,2例单纯疱疹病毒(HSV)和1例甲型肝炎病毒(HAV)。未发现药物相关因素。分别进行个体化治疗,重点在早期发现和联合抗病毒治疗,适度地联合免疫化疗(HLH-94或环孢素A)可以获得及时的缓解。1例SOJIA患儿严重肝损伤经连续性血液滤过/肾脏替代治疗联合免疫化疗获得迅速缓解。2例SOJIA患儿死于严重的内脏出血。结论MAS不仅见于SOJIA,还可以发生在儿科其他风湿性疾病的任何阶段,其发生率可能要高于以往的认识。感染可能是主要的触发因素。适时的免疫净化联合免疫化疗有益于严重肝损伤的恢复。
The purpose of the diagnosis and treatment of macrophage activation syndrome (MAS) has not yet been unified, summarize the clinical data of 6 cases of children with MAS in our hospital to provide reference for clinical treatment. Methods The clinical data of patients with rheumatic diseases complicated with MAS from 1998 to 2004 in our hospital were retrospectively analyzed. Results A total of 6 patients (4 males and 2 males) met the diagnostic criteria of MAS. Four cases of juvenile idiopathic arthritis (SOJIA) and two diagnosed systemic lupus erythematosus and lupus nephritis were diagnosed before MAS occurred. In all cases, the disease status was in a low activity stage when MAS was diagnosed. The main clinical features were: peak fever (5/6) or moderate fever (1/6), hepatosplenomegaly (6/6), lymphadenopathy (6 / 6), 3 (6/6) reduction in peripheral blood, significant liver injury (6/6), disseminated intravascular coagulation (2/6) and central nervous system dysfunction (3/6). Hypofibrinogenemia, elevated liver enzymes, and hypertriglyceridemia persisted. 3 cases of bone marrow smear visible phagocytosis and plasma cell island, the other 3 cases did not do this check. Three children were confirmed as having an infection, two herpes simplex virus (HSV) and one hepatitis A virus (HAV). No drug-related factors were found. Individualized treatment, with emphasis on early detection and combination antiviral therapy, moderate combination of immunotherapy (HLH-94 or cyclosporine A) can be timely relief. A case of severe liver injury in children with SOJIA by continuous hemofiltration / renal replacement therapy combined with immunotherapy to obtain rapid relief. Two children with SOJIA died of severe visceral hemorrhage. Conclusion MAS is not only found in SOJIA but can occur at any stage of pediatric rheumatic disease and its incidence may be higher than previously recognized. Infection may be the main trigger. Timely immunosuppressive combined immunotherapy is beneficial for the recovery of severe liver injury.