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目的探讨儿童感染继发噬血细胞综合征的诊治及预后。方法对1例多病原感染继发噬血细胞综合征的患儿进行临床分析,并复习相关文献。结果该例患儿以肺炎住院,反复高热、存在粒细胞缺乏及贫血,骨髓中发现噬血细胞、NK细胞活性降低、可溶性IL-2R(CD25)≥2 400 U/ml,噬血细胞综合征相关基因突变分析报告未提示有病理意义的突变,感染相关性噬血细胞综合征诊断成立。予丙种球蛋白及地塞米松治疗。后因半乳甘露聚糖(GM)试验显著升高,监测胸部影像学检查逐渐出现真菌性肺炎表现,加用抗真菌治疗,病情渐趋好转。入院后多次呼吸道分泌物直接免疫荧光法7项病毒抗原检测均无阳性发现,血清学检查结果也不支持EB病毒、柯萨奇病毒、埃柯病毒、HIV、巨细胞病毒(CMV)、风疹病毒、单纯疱疹病毒I、弓形体及肺炎支原体感染,后经PCR、实时PCR及Luminex技术平台检测证实存在甲型H1N1流感病毒及呼吸道合胞病毒B亚型合并感染。结论对感染相关性噬血细胞综合征,除对症、支持治疗外,按HLH-2004方案,同时兼顾并发症的治疗。预后可能取决于有无基础疾病、诊断的早晚及是否出现严重并发症等。
Objective To investigate the diagnosis and treatment of children with secondary hemophagocytic syndrome infection and prognosis. Methods One case of multi-pathogen infection secondary hemophagocytic syndrome in children clinical analysis, and review the relevant literature. Results The patients were admitted to hospital with pneumonia, recurrent fever, presence of agranulocytosis and anemia, hemophagocytic cells found in the bone marrow, decreased NK cell activity, soluble IL-2R (CD25) ≥ 2400 U / ml, hemophagocytic syndrome-related genes Mutational analysis report did not prompt pathological changes, infection associated hemophagocytic syndrome diagnosis established. To gamma globulin and dexamethasone treatment. After the galactomannan (GM) test was significantly higher, monitoring of chest imaging studies gradually showed fungal pneumonia, plus antifungal therapy, the condition is getting better. After admission, respiratory secretions were detected by direct immunofluorescence. None of the 7 virus antigens tested showed positive results. Serological tests did not support Epstein-Barr virus, Coxsackie virus, Erkovirus, HIV, cytomegalovirus (CMV), rubella Virus, herpes simplex virus I, toxoplasma and mycoplasma pneumoniae infection, confirmed by PCR, real-time PCR and Luminex technology platform to confirm the presence of influenza A (H1N1) and respiratory syncytial virus subtype B infection. Conclusion In addition to symptomatic and supportive treatment of infection with hemophagocytic syndrome, HLH-2004 regimen was used to treat complications. Prognosis may depend on the presence or absence of underlying disease, the diagnosis of sooner or later and whether there are serious complications.