多病原感染继发噬血细胞综合征1例

Hemophagocytic syndrome secondary to infection caused by multiple pathogens in a child

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 explore the diagnosis, treatment and prognosis of hemophagocytic syndrome （HPS） secondary to infection in children. Methods The clinical data of a child with HPS secondary to multiple pathogens was analyzed, and the related literatures were reviewed. Results The girl was admitted because of pneumonia, recurrent high fever, accompanied with neutropenia and anemia. Given the combination of hemophagocytosis in the bone marrow, decreased natural killer （NK） cell activity, elevated soluble IL-2 receptor （IL-2R） levels, no evidence of gene mutation related with HPS, the diagnosis of infection-related HPS was made. The child was given IVIG and dexamethasone intravenously, followed by antifungal therapy due to highly elevated Galactomannan （GM） assay and findings of aspergillus pneumonia of her Chest Computed Tomography later. The patient was getting recoved gradually. No positive results were found through multiple ways to detect the virus pathogeny, including detection of multi-virus antigens through direct immunofluorescence technique and multiple IgM antibody detection. Eventually both H1N1 virus and RSV subtype B were detected from lower respiratory tract secretion by PCR, real-time PCR and kits for the Luminex Corp. Conclusion For infectionrelated HPS, in addition to symptomatic and support treatment, following the HLH-2004 protocols, measures should be taken to manage the complications meanwhile. Prognosis depends on whether there is underlying disease exists, being promptly diagnoses or delayed for the diagnosis and whether severe complications are developed.