儿童巨噬细胞活化综合征23例临床分析

Clinical analysis on 23 cases of childhood macrophage activation syndrome

目的:分析巨噬细胞活化综合征患儿的临床及实验室检查特点,以提高对该病的认识。方法:回顾性分析我院收治的23例巨噬细胞活化综合征患儿的临床表现、早期特征、实验室指标、治疗及转归。结果:23例患儿中男11例,女12例,平均年龄7.1岁;基础疾病1例为川崎病,2例为系统性红斑狼疮,余20例均为幼年特发性关节炎全身型;15例患儿以高热不退,7例患儿以黄疸、重症肝炎,1例患儿以溶血性贫血为首发表现。所有患儿均有肝脾或(和)淋巴结进行性增大、血液系统受累,4例有中枢神经系统症状,5例表现易出血,4例呼吸系统受累(支气管肺炎、呼吸衰竭),8例有消化系统症状,心脏、肾脏受累各1例,1例合并腮腺炎。实验室检查均有外周血象至少一系降低,血清转氨酶增高、乳酸脱氢酶增高、红细胞沉降率降低、血清铁蛋白增高、凝血常规异常、甘油三酯升高及骨髓中发现吞噬血细胞。所有患儿予以个体化治疗,应用甲基强的松龙、静脉丙种球蛋白冲击+静脉或口服环孢霉素A治疗效果较好。经过治疗后21例好转,2例死亡。结论:巨噬细胞活化综合征不仅见于幼年特发性关节炎全身型,还可见于其他风湿免疫性疾病(川崎病、系统性红斑狼疮),病情凶险,可以造成全身多脏器损害,有时发病以重症肝炎、溶血性贫血等为首发表现而不伴有持续高热,予静脉丙种球蛋白、甲基强的松龙冲击及环孢素A治疗可以提高缓解率,早期诊断、早期治疗是提高生存率的关键。

To analyze the clinical and laboratory characteristics of macrophage activation syndrome （MAS） in children. Methods： Clinical data （ the clinical features, early manifestation, laboratory characteristics,treatment, and outcome） of 23 cases of MAS, who came from our hospital, were retrospectively analyzed. Results： The average age of 23 MAS children （including 11 male and 12 female ） was 7.1-year-old. Underlying diseases were Kawasaki disease （ 1 case）, systemic lupus erythematosus （2 cases） and systemic type juvenile idiopathic arthritis （20 cases）. The first clinical manifestations were persisting high fever （15 cases）, jaundice and fulminate hepatitis （7 cases）, hemolytic anemia （1 case）. All the MAS children manifested the progressive enlargement of both liver and spleen, or （and） lymph nodes associated with blood system involvement, 4 cases with central nervous system dysfunction, 5 cases with easy bleeding, 4 cases with respiratory system dysfunction such as bronchial pneumonia or respiratory failure, 8 cases with digestive system dysfunction, 1 case with cardiac disease,1 case with renal disease and 1 case with mumps. One to three series of peripheral blood cells and erythrocyte sedimentation rate decreased. While the serum transaminase, lactate dehydrogenase, serum ferritin and triglycerides increased. Other dysfunctions were coagulopathy and phagocytic blood cells in bone marrow. The treatment protocols were set individually. Application of methylprednisolone and intravenous gamma globulin therapy with cyclosporin A given intravenously or orally were very effective. In spite of 2 cases died, others got recovered. Conclusions： MAS is not only seen systemic juvenile idiopathic arthritis, but also seen in other rheumatic and immunological diseases （ Kawasaki disease, systemic lupus erythematosus ）. MAS is a dangerous disease and can cause systemic multiple organ damage. Sometime severe hepatitis and hemolytic anemia is the first symptom instead of persistent fever. Treatment with intravenous gamma globulin, methylprednisolone and cyclosporine A can improve the remission rate. Early diagnosis and treatment is key to improve prospects of survival.