儿童重症社区获得性肺炎触发的噬血细胞综合征17例临床分析  

Hemophagocytic lymphohistiocytosis triggered by severe community-acquired pneumonia in children:a clinical analysis of 17 cases

作　　者：辛琪[1] 孙婷婷[1] 徐刚[1] XIN Qi;SUN Tingting;XU Gang(Department of Pediatrics,Shengjing Hospital of China Medical University,Shenyang 110004,China)

机构地区：[1]中国医科大学附属盛京医院儿内科,沈阳110004

出　　处：《中国医科大学学报》2023年第1期62-67,共6页Journal of China Medical University

摘　　要：目的 探讨儿童重症社区获得性肺炎(CAP)触发的噬血细胞综合征(HLH)的临床特点。方法 回顾性分析17例重症CAP触发的HLH临床资料,总结临床特征,分析影响患者预后的不良因素。结果 17例患者年龄1.2~12.1岁,男6例,女11例;均有发热,以皮疹(47.1%)、脾大(70.6%)、低氧血症(82.4%)和呼吸衰竭(41.2%)为主要表现,肺内外并发症多见。混合病原感染13例(76.5%),病毒感染12例(70.6%)。1例发生STXBP2杂合突变,考虑为重症CAP触发的原发性HLH。治疗后随访存活组12例(70.6%),死亡组5例(29.4%),对比分析2组实验室指标发现,血小板计数<80×109/L是影响重症CAP触发HLH预后的不良因素(P=0.009)。结论 重症CAP触发的HLH病情危重,死亡率高,常见于以病毒感染为主的混合感染,应注意筛查是否为原发性,血小板减少可能与不良预后相关。Objective To investigate the clinical characteristics of hemophagocytic lymphohistiocytosis(HLH) triggered by severe community-acquired pneumonia(CAP) in children. Methods The clinical data of 17 patients with HLH triggered by severe CAP were retrospectively analyzed. The clinical characteristics were summarized,and adverse prognostic factors were analyzed. Results The age of the 17 patients(6 males,11 females) ranged from 1.2 year and 12.1 years. The main manifestations were rash(47.1%),splenomegaly(70.6%),hypoxemia(82.4%),and respiratory failure(41.2%). Intrapulmonary and extrapulmonary complications were common. A total of 13 cases(76.5%) had mixed infections,of which 12(70.6%) were caused by virus infection. One case had a heterozygous mutation of STXBP2,which was considered primary HLH triggered by severe CAP. After treatment,12 patients(70.6%) in the survival group and 5(29.4%) in the death group were followed up. Comparative analysis of laboratory indicators between the two groups showed that a platelet count less than 80×10~9/L was an adverse factor affecting the prognosis of severe CAP-triggered HLH(P = 0.009). Conclusion Severe CAP-triggered HLH is a critical illness with high mortality. Mixed infections mainly caused by virus infection are common. Care should be taken in screening for primary HLH,as thrombocytopenia may be associated with a poor prognosis.

关 键 词：重症社区获得性肺炎 噬血细胞综合征 预后 儿童 

分 类 号：R725.5[医药卫生—儿科]