儿童继发性噬血细胞性淋巴组织细胞增生症5例临床分析

Clinical analysis of five cases with pediatric secondary hemophagocytic lymphohistiocytosis

目的分析不同病因儿童继发性噬血细胞性淋巴组织细胞增生症(HLH)的临床表现、实验室检查特点及治疗情况,探讨儿童HLH的分层诊断和治疗,提高儿科临床医师对该病的认识。方法回顾性分析2018年1月至2019年12月在首都医科大学附属儿童医院血液肿瘤中心诊治的5例继发性HLH患儿的临床资料,分析HLH原发病的诊断及后续治疗情况。结果5例患儿中男3例,女2例;中位发病年龄1.5(0.5~12.7)岁。发病初期均表现为发热伴血细胞减低,入院后完善相关检查,诊断为HLH。原发病分别为淋巴瘤、肝炎相关再生障碍性贫血、朗格汉斯细胞组织细胞增生症、系统性自身炎症性疾病和先天遗传代谢病。治疗上除控制HLH外,原发病的治疗至关重要。结论儿童HLH是一类综合征,病因复杂,可以继发于多种疾病。因此对于儿童HLH,需注意病因学方面的鉴别诊断,在发病初期尽可能完善相关检查,明确病因,根据病因进行分层治疗。

Objective To analyze the clinical manifestations,laboratory examination characteristics and treatment of secondary hemophagocytic lymphohistiocytosis(HLH)in children with different etiologies,and to further explore the stratified diagnosis and treatment of pediatric secondary HLH,in an attempt to improve pediatricians′understanding of this disease.Methods The clinical data of 5 children with secondary HLH treated at Beijing Children′s Hospital,Capital Medical University from January 2018 to December 2019 were retrospectively analyzed,to explore the diagnosis and subsequent treatment of the primary HLH.Results Five patients(including 3 boys and 2 girls)had a median onset age of 1.5(0.5-12.7)years.At the early stage of the disease,all patients had fever and hemocytopenia.After admission,HLH-related examinations were performed and secondary HLH was diagnosed.The underlying primary diseases were lymphoma,hepatitis-associated aplastic anemia,Langerhans cell histiocytosis,systemic autoinflammatory disease and hereditary metabolic disease.Except for controlling HLH activation,the treatment of primary disease was also of great significance.Conclusions Pediatric HLH is a group of syndrome with complex underlying diseases.Therefore,pediatricians should pay more attention to the stratified diagnosis of underlying primary diseases of pediatric secondary HLH.Related laboratory tests should be performed as soon as possible to determine the etiology after HLH is diagnosed.