肺炎支原体相关性噬血细胞综合征11例病例系列报告

目的：探讨肺炎支原体相关性噬血细胞综合征（MP-HPS）的临床特点、诊断、治疗及转归。方法：回顾性分析2013年6月1日至2018年12月31日深圳市儿童医院收治的MP-HPS患儿的临床资料。结果：11例MP-HPS患儿的中位年龄为3岁3个月（7个月至9岁7个月）,<3岁5例,男6例、女5例。均有发热,咳嗽8例,腹泻3例,精神差1例;均有肝大,脾大6例。均表现为不同程度地血细胞减少、血清铁蛋白升高（≥500 μg·L-1）和乳酸脱氢酶不同程度地升高;骨髓象提示均有噬血细胞现象;肝功能异常8例,高甘油三酯9例,低纤维蛋白原血症6例,NK细胞活性降低8例;11例均经MP-IgM和/或MP-DNA检查证实MP感染,合并细菌和真菌感染各2例,未检测到其他病毒感染。胸部X线和CT显示,11例均有肺炎表现,合并胸腔积液7例,合并纵膈气肿和颈胸部皮下气肿1例。7例行支气管镜检查,塑形性支气管炎2例。治疗和预后：均予大环内酯类联合其他抗生素治疗,使用糖皮质激素和丙种球蛋白各10例、环孢素4例,环孢素+依托泊苷1例;7例入住PICU,其中气管插管5例;好转出院9例,死亡2例。结论:MP-HPS应早期明确诊断,积极治疗,及时阻断细胞因子风暴,以改善患儿的预后。

11 cases of Mycoplasma pneumoniae-associated hemophagocytic syndrome: A case series report

Objective:To investigate the clinical characteristics, diagnosis, treatment and prognosis of mycoplasma pneumoniae-associated hemophagocytic syndrome (MP-HPS). Methods:The clinical data of MP-HPS children admitted to Shenzhen Children's Hospital between June 1, 2013 and December 31, 2018 were retrospectively analyzed. Results：The median age of 11 patients with MP-HPS was 3 years and 3 months.There were 5 cases aged younger than 3 years, 6 males and 5 females. Clinical manifestations included fever in 11 cases, cough in 8 cases, diarrhea in 3 cases, and poor mental health in 1 case. All patients had large liver, and large spleen was found in 6 cases. Laboratory examination results showed all of 11 patients had different degrees of hemocytopenia, elevated serum ferritin (≥ 500 μg·L-1) and lactate dehydrogenase.Bone marrow images suggested hemophagocytosis in 11 cases. There were 8 cases of abnormal liver function, 9 cases of high triglyceride, 6 cases of low fibrinogenemia, and 8 cases of decreased NK cell activity. MP infection was confirmed by MP-IgM MP-DNA tests in all 11 cases with 2 cases of combined bacterial infections and 2 cases of fungal infections. No other viral infections were detected. Imaging examination (chest X-ray and chest CT) results showed all of 11 cases presented pneumonia, 7 cases were complicated with pleural effusion, and 1 case was complicated with mediastinal emphysema and subcutaneous emphysema of the neck and chest. Seven cases underwent bronchoscopy and 2 cases had plastic bronchitis. Macrolides were combined with other antibiotics. Glucocorticoid and gamma globulin were used in 10 patients, immunosuppressant (cyclosporin) was used in 4 patients, and cyclosporin + etoposide was used in 1 patient. Among the 7 patients admitted to PICU, 5 were intubated. Nine cases were discharged after improvement and 2 cases died. Conclusion:MP-HPS should be diagnosed early and treated actively. Cytokine storm should be blocked in time to improve the prognosis of children.