ISSN 1674-3865  CN 21-1569/R
主管：国家卫生健康委员会
主办：中国医师协会
　　　辽宁省基础医学研究所
　　　辽宁中医药大学附属医院
中国中西医结合儿科学

中国中西医结合儿科学 ›› 2021, Vol. 13 ›› Issue (3): 234-236.

• 临床研究 • 上一篇    下一篇

儿童噬血细胞综合征24例临床分析

目的: 探讨噬血细胞综合征的临床特点、诊断、个体化治疗及预后。
方法: 回顾性分析2016年9月至2019年12月我院收治的24例儿童噬血细胞综合征的临床资料。
结果: 高热、肝脾肿大是最常见的临床表现，亦有中枢神经系统受累及急性肝功能衰竭为首发临床表现的噬血细胞综合征。全血细胞减少（29.2%）、凝血功能异常（83.3%）、甘油三酯增多（45.8%）、肝功能损害（87.5%）、乳酸脱氢酶增高（100%）、C反应蛋白升高（70.8%）、钙离子降低（25%）、钠离子降低（66.7%）、铁蛋白升高（87.5%）、自然杀伤细胞活性降低（91.7%）、可溶性白细胞介素-2受体水平升高（100%）是本组病例实验室特点。骨髓象：23例均可见成熟组织细胞和噬血细胞。
结论: 噬血细胞综合征临床表现复杂多样，临床医生应早期认症，早期诊断，并积极寻找原发病因，及早对患者进行个体化治疗是取得良好疗效的关键。   

  1. 116000辽宁大连，大连市儿童医院血液肿瘤科
  • 出版日期:2021-06-25 发布日期:2021-12-15
  • 通讯作者: 边爽，E-mail：bianshuang1985@163.com
  • 作者简介:边爽（1985-），女，医学硕士，主治医师。研究方向：儿童血液肿瘤的诊治

Clinical analysis of hemophagocytic syndrome in children

Objective: To investigate the clinical features，diagnosis，individualized treatment and prognosis of hemophagocytic syndrome(HPS).#br# Methods: A retrospective study was carried out to analyze the clinical data of 24 HPS cases treated in our hospital from Sep. 2015 to Nov. 2018.#br# Results: The most common clinical manifestations were fever and splenomegaly.There were also central nervous involvement and acute liver failure as the first clinical manifestations of HPS.The features of laboratory data included cytopenia(29.2%)，coagulation disorder(83.3%)，high level of triglyceride(45.8%)，hepatic function damage(87.5%)，elevated lactate dehydrogenase(100%)，elevated C reactive protein(70.8%),calcium ion(25%)，reduced sodion(66.7%)，elevated ferritin(87.5%)，reduced activity of NK cells(91.75%)，and elevated level of sCD 25(100%); hemophagocytosis and histiocytes could be seen in bone marrow of 23 cases.#br# Conclusion: HPS has diverse clinical manifestations;therefore,good results can be obtained by early identification,early diagnosis,and trying to find the primary cause in order to perform individualized treatment.   

  1. Dalian Children′s Hospital，Dalian 116000，China
  • Online:2021-06-25 Published:2021-12-15

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摘要： 目的: 探讨噬血细胞综合征的临床特点、诊断、个体化治疗及预后。
方法: 回顾性分析2016年9月至2019年12月我院收治的24例儿童噬血细胞综合征的临床资料。
结果: 高热、肝脾肿大是最常见的临床表现，亦有中枢神经系统受累及急性肝功能衰竭为首发临床表现的噬血细胞综合征。全血细胞减少（29.2%）、凝血功能异常（83.3%）、甘油三酯增多（45.8%）、肝功能损害（87.5%）、乳酸脱氢酶增高（100%）、C反应蛋白升高（70.8%）、钙离子降低（25%）、钠离子降低（66.7%）、铁蛋白升高（87.5%）、自然杀伤细胞活性降低（91.7%）、可溶性白细胞介素-2受体水平升高（100%）是本组病例实验室特点。骨髓象：23例均可见成熟组织细胞和噬血细胞。
结论: 噬血细胞综合征临床表现复杂多样，临床医生应早期认症，早期诊断，并积极寻找原发病因，及早对患者进行个体化治疗是取得良好疗效的关键。

关键词: 噬血细胞综合征, 临床特征, 个体化治疗

Abstract: Objective: To investigate the clinical features，diagnosis，individualized treatment and prognosis of hemophagocytic syndrome(HPS).
Methods: A retrospective study was carried out to analyze the clinical data of 24 HPS cases treated in our hospital from Sep. 2015 to Nov. 2018.
Results: The most common clinical manifestations were fever and splenomegaly.There were also central nervous involvement and acute liver failure as the first clinical manifestations of HPS.The features of laboratory data included cytopenia(29.2%)，coagulation disorder(83.3%)，high level of triglyceride(45.8%)，hepatic function damage(87.5%)，elevated lactate dehydrogenase(100%)，elevated C reactive protein(70.8%),calcium ion(25%)，reduced sodion(66.7%)，elevated ferritin(87.5%)，reduced activity of NK cells(91.75%)，and elevated level of sCD 25(100%); hemophagocytosis and histiocytes could be seen in bone marrow of 23 cases.
Conclusion: HPS has diverse clinical manifestations;therefore,good results can be obtained by early identification,early diagnosis,and trying to find the primary cause in order to perform individualized treatment.

Key words: Hemophagocytic syndrome, Clinical characteristics, Individualized treatment