朗格汉斯细胞组织细胞增生症合并噬血细胞综合征4例报道并文献复习

Langerhans cell histiocytosis complicated with hemophagocytic lymphohistiocytosis:Four cases report and literature review

目的 分析儿童朗格汉斯细胞组织细胞增生症(LCH)合并噬血细胞综合征(HLH)的临床特征并探讨影响其预后的因素,提高对儿童LCH合并HLH的认识。方法 回顾性分析2019年11月—2021年7月湖南省儿童医院血液肿瘤科收治的4例LCH合并HLH患儿的临床资料,并复习相关文献。结果 4例患儿中女性3例,男性1例,截止至随访时间4例患儿均存活。诊断LCH时患儿的平均年龄2.56±0.11岁,均出现发热,脾大及骨髓噬血现象,受累部位皆为多系统伴危险器官受累。4例患儿在诊断HLH时均进行细胞因子检测,所有患儿IL-10和IFN-Υ均明显升高。结论 LCH和HLH同属于组织细胞疾病,MS-LCH伴危险器官受累的患儿,当年龄<2岁,sIL-2R升高、铁蛋白水平不高时更容易并发HLH,当LCH病例出现HLH相关细胞因子谱改变时有助于该两种临床病变的鉴别诊断。

Objective To analyze the clinical features and prognostic factors of children with Langerhans cell histiocytosis(LCH)complicated with hemophagocytic lymphohistiocytosis(HLH),so as to improve the understanding of LCH complicated with HLH in children.Methods Clinical data of 4 children with LCH complicated with HLH admitted to Hunan Children′s Hospital from November 2019 to July 2021 were analyzed retrospectively,and the relevant literature were reviewed.Results Among the 4 cases,there were 3 females and 1 male.As of the last follow-up,4 cases were survived.The average age of the children at the time of diagnosis of LCH was 2.56±0.11 years old.Fever,splenomegaly and bone marrow phagocytosis occurred in all the patients.The involved sites were all multisystem with risk organ involvement.Cytokines were detected in 4 children when they were diagnosed with HLH,and the levels of IL-10 and IFN-γwere significantly increased in all children.Conclusions LCH and HLH are histiocytic diseases.Multisystem LCH(MS-LCH)patients with risk organ involvement are more likely to complicated with HLH when they are younger than 2 years old,with elevated sIL-2R and low ferritin levels.The change of HLH-related cytokine spectrum in LCH cases is helpful for the differential diagnosis of the two clinical lesions.