肺炎支原体相关性噬血细胞综合征三例并文献复习

Analysis of 3 cases with Mycoplasma pneumoniae-associated hemophagocytic syndrome and review of literature

目的 分析肺炎支原体相关性噬血细胞综合征（MP-HLH）的临床特点.方法 对2013年6月-2013年7月深圳市儿童医院收治的3例MP-HLH患儿的临床表现、实验室检查、影像学改变、治疗经过、转归进行回顾性总结分析,并复习相关文献.结果 3例MP-HLH患儿男2例,女1例,年龄分别为1岁、3岁和6岁,均无基础性疾病.均以发热、咳嗽为主要表现起病.明确为难治性肺炎支原体肺炎,病程在第3周开始出现中性粒细胞[（0.08 ～ 0.68）×109/L]、红蛋白[（79～103）g/L]、血小板[（64～157）×109/L]、血浆纤维蛋白原[（1.3～1.5）g/L]下降,乳酸脱氢酶[（1 170～1 285） U/L]、血清铁蛋白[（936.7 ～39 789.0）μg/L]增高.2例T细胞减少,1例NK细胞活性显著下降.骨髓增生活跃,并出现吞噬血细胞现象.CT均发现肺内实变伴胸腔积液,2例入住PICU,行气管插管和机械通气.3例均使用可弯曲支气管镜检查,2例吸出树样支气管管型.3例均予以大环内酯类药物联合其他抗生素抗感染,糖皮质激素及丙种球蛋白联合治疗,其中1例给予地塞米松[10mg/（m2·d）]、环孢霉素[6 mg/（kg·d）]、依托泊苷[150 mg/（m2·d）]化疗.最终2例治愈出院,1例死亡.国内外文献报道的18例患儿中,国外患儿在1～2周内诊断并开始激素治疗,治愈10例,死亡2例,分别死于出血倾向及脑膜脑炎.国内患儿在2周～1个月后诊断及治疗,治愈5例,死亡1例,死于重症肺炎.结论 儿童MP-HLH是一种罕见并发症,起病急,进展快,病死率高.早期予以激素治疗预后良好,避免免疫瀑布启动是治疗成功的关键.死亡可能与诊断过晚或出现其他严重合并症有关.

Objective To analyze the clinical characteristics of Mycoplasma pneumoniae-associated hemophagocytic syndrome （MP-HLH）.Method A retrospective investigation of the clinical manifestation,laboratory test,imagelogy,clinical course and outcome of 3 cases with MP-HLH seen between June 2013 and July 2013 in Shenzhen Children＇s Hospital,and review of relevant literature were conducted.Result Of the 3 cases of MP-HLH,2 were males,one was female,the ages were 1 year,3 years and 6 years,respectively.They had no underlying disease previously.All the 3 cases had onset of fever,cough as main symptoms.Diagnosis of refractory Mycoplasma pneumoniae pneumonia was made,which was accompanied by decreased neutrophils [（0.08-0.68） × 109/L],hemoglobin [（79-103） g/L],platelet [（64-157） × 109/L],plasma fibrinogen [（1.3-1.5）g/L],lactate dehydrogenase [（1 170-1 285） U/L] and increased serum ferritin [（936.7-39 789.0） μg/L] in the third week of course.In two cases the T lymphocytes decreased,and the NK cell activity decreased significantly in one.Bone marrow cytology showed prompted bone marrow hyperplasia,and the phenomenon of phagocytosed blood cells.CT scan was performed for all the cases and consolidation with pleural effusion were shown.Two cases were admitted to PICU,and required endotracheal intubation and mechanical ventilation.Flexible bronchoscopy and bronchial lavage were performed and bronchial cast was found in two cases.All of them were treated with macrolide combined with other antibiotics,glucocorticoids and gamma globulin combination therapy,including one case given dexamethasone [10 mg/ （m2 · d）],cyclosporine[6 mg/（kg· d）],etoposide [150 mg/ （m2 · d）]chemotherapy.Two cases were cured,and 1 case died.The authors summarized the 18 cases reported in domestic and foreign literature.Foreign children were diagnosed and treated with steroids in 1-2 weeks,and 10 cases were cured,and 2 cases died.They died of massive hemorrhage and meningoencephalitis,and domestic children were diagnosed and treated within two to 4 weeks after onset,5 cases were cured,one case died of severe pneumonia.Conclusion MP-HLH is a rare disease in children,and had acute onset,rapid progression and high mortality.Early treatment with steroids was associated with a good prognosis,the key to successful treatment is early diagnosis and treatment,avoiding the immune cascade.Too late a diagnosis or development of serious complications may lead to death.