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目的总结3例系统性红斑狼疮(SLE)-巨噬细胞活化综合征(MAS)患儿的临床资料,为儿童SLE-MAS诊治提供参考。方法回顾性分析中国医科大学附属第一医院儿科2008—2014年SLE-MAS患儿临床资料。结果本组共3例,男女比例为2∶1,2例在疾病早期即有相关临床症状,表现为发热、血象异常、高血清铁蛋白、肝功能及凝血异常等。所有确诊病例均为青春期患儿。主要临床症状包括高热(3/3)、肝脾受累(3/3)、出血(3/3)、肾脏受累(3/3)、心脏受累(2/3)、肺脏受累(1/3)及中枢神经系统症状(1/3)。主要实验室异常包括外周血三系减少(3/3)、高血清铁蛋白(3/3)、高甘油三脂(3/3)、低白蛋白(3/3)、天冬氨酸氨基转移酶(AST)增高(2/3)、乳酸脱氢酶(LDH)增高(2/3)、NK细胞活性减低(2/3)、低纤维蛋白原(1/3)、低钠血症(1/3)、血沉正常或减少(0/3),3例患儿行骨髓穿刺术均提示巨噬细胞活化,其中有2例SLE患儿初次骨穿未见特征性改变,病程进展中复查不同部位骨髓,可见较多嗜血细胞。3例患儿经激素、丙种球蛋白及环孢素A治疗后,1/3病情明显改善,2/3控制不佳,放弃治疗,其中1例给予血浆置换共5次,疗效不确切。结论 MAS可发生于SLE各个阶段,甚至可作为首发症状,常常以高热为突出表现,预后不佳,治疗需遵循个体化,激素及免疫制剂治疗有效,静脉输注丙种球蛋白及血浆置换有利于疾病的恢复。
Objective To summarize the clinical data of 3 patients with systemic lupus erythematosus (SLE) -activated macrophage syndrome (MAS) and provide references for the diagnosis and treatment of SLE-MAS in children. Methods The clinical data of children with SLE-MAS from 2008 to 2014 in the First Affiliated Hospital of China Medical University were retrospectively analyzed. Results A total of 3 patients in this group were male and female with a ratio of 2: 1. Two patients had clinical symptoms early in the disease, which manifested fever, abnormal blood, high serum ferritin, hepatic dysfunction and coagulation. All confirmed cases were adolescent children. The main clinical symptoms include high fever (3/3), liver and spleen involvement (3/3), bleeding (3/3), kidney involvement (3/3), heart involvement (2/3), lung involvement (1/3) And central nervous system symptoms (1/3). Major laboratory abnormalities include a decrease in peripheral blood three lines (3/3), high serum ferritin (3/3), high triglyceride (3/3), low albumin (3/3), aspartate amino (2/3), elevated lactate dehydrogenase (LDH) (2/3), reduced NK cell activity (2/3), low fibrinogen (1/3), hyponatremia (1/3), normal or decreased erythrocyte sedimentation rate (0/3). Three cases of children underwent bone marrow aspiration and activated macrophages. There was no characteristic change in the initial bone formation of 2 children with SLE, Review different parts of the bone marrow, showing more bloodthirsty cells. Three patients with hormone, gamma globulin and cyclosporin A treatment, 1/3 condition improved significantly, 2/3 poor control, to give up treatment, including 1 case of plasma exchange for a total of 5 times, the effect is not exact. Conclusion MAS can occur in all stages of SLE, even as the first symptom, often with high fever as the outstanding performance, the prognosis is poor, the treatment should follow the individualized, hormone and immunotherapy effective treatment, intravenous gamma globulin and plasma exchange is conducive Recovery of the disease.