小儿噬血细胞性淋巴组织细胞增生症42例临床分析

A Clinical Analysis of 42 Children with Hemophagocytic Lymphohistiocytosis

目的探讨小儿噬血细胞性淋巴组织细胞增生症(Hemophagocytic lymphohistiocytosis,HLH)的病因、临床特点、诊断、治疗及预后情况。方法回顾分析42例小儿HLH的临床资料。结果 42例中男26例,女16例,临床症状以持续高热、肝脾肿大为突出表现,同时部分患儿合并呼吸系统、中枢神经系统、循环系统、泌尿系统等其它脏器受累;42例中感染相关性26例;非感染相关性5例;剩下11例病因不明;根据HLH-94或HLH-2004方案治疗后,好转或治愈19例,死亡13例,失访10例。采用流式细胞术检测了25例患儿外周血CD3+/CD4+、CD3+/CD8+、CD3-/CD19+、NK(CD3-/CD16+/CD56+)细胞的表达水平,结果示患儿CD4+、NK(CD3-/CD16+/CD56+)细胞百分率明显降低(P<0.05),CD8+、B(CD19+)细胞百分率明显升高(P<0.05)。结论小儿HLH病因复杂,临床表现多样,病死率较高,外周血淋巴细胞亚型的改变可能与其发病及病情严重程度密切相关。

Objective To analyze the etiology,clinical manifestations,diagnosis,therapy and prognosis of hemophagocytic lymphohistiocytosis（HLH）in pediatric patients.Methods Forty-two pediatric patients with HLH were retrospectively reviewed.Results In 26 boys and 16 girls,the clinical manifestations were featured with prolonged high fever and hepatosplenomegaly;in addition,the organs of the respiratory system,central nervous system,circulatory system and urinary tract were concurrently affected.Among the 42 cases,26 cases were infection-related,5 non-infection related,and 11 unknown.Treated as per HLH-94 or HLH-2004 scheme,19 cases were cured or improved,13 cases died and 10 cases failed to continue follow-up.The expression levels of CD3＋/CD4＋,CD3＋/CD8＋,CD3-/CD19＋and NK cells（CD3-/CD16＋/CD56＋）in peripheral blood from these patients were determined by flow cytometry.The results showed significant decrease in CD4＋,NK cells（CD3-/CD16＋/CD56＋,P0.05）and significant increase in CD8＋,B（CD19＋,P0.05）in these patients.Conclusion HLH is featured with complicated etiology,varying clinical manifestations and relatively high mortality.Changes in lymphocyte subsets in the peripheral blood of the patients are related to the onset and severity of HLH.