目的 探讨噬血细胞性淋巴组织细胞增生症（HLH）的临床特征及预后因素。方法 回顾分析2015年1月至2020年10月收治的175例HLH患儿的临床资料,比较存活组和死亡组的临床特征,采用log-rank检验及COX比例风险回归模型进行生存分析。结果 175例HLH患儿中男91例、女84例,中位年龄2.0（1.0~8.0）岁,以婴幼儿期起病最常见（54.3%）。原发性HLH 9例,继发性HLH 166例。继发性HLH中以感染相关性HLH最常见（66.9%）。临床以发热、脾大为突出表现,血清乳酸脱氢酶（LDH）升高最为显著。与存活组相比,死亡组患儿白细胞计数和血红蛋白较低,活化部分凝血活酶时间、凝血酶原时间较长,纤维蛋白原（FIB）较低,中枢神经系统受累率较高,差异均有统计学意义（P<0.05）。中枢神经系统受累、FIB≤1.5 g/L是影响患儿总生存期的独立危险因素（P<0.05）。结论 HLH多见于婴幼儿,临床表现以发热最多见,血清LDH大多异常升高。中枢神经系统受累及FIB水平对初诊HLH患儿疗效评估及预后判断具有重要意义。

Objective To investigate the clinical characteristics and prognostic factors of hemophagocytic lymphohistosis (HLH). Methods The clinical data of 175 children with HLH admitted from January 2015 to October 2020 were retrospectively analyzed. Clinical manifestations of the patients of HLH in the survival group and the deceased group were compared. Log-rank test and COX proportional riskregression model were used for survival analysis. Results Among the 175 cases, 91 were male and 84 were female, with a median age of 2.0 (1.0-8.0) years, and the most common onset age was in infancy (54.3%). There were 9 cases of primary HLH and 166 cases of secondary HLH. Infection-associated HLH was the commonest in secondary HLH (66.9% of them were EBV infection). The clinical manifestations were persistent fever (99.4%) and splenomegaly (72%). Laboratory examination showed that elevated serum LDH was the most significant (97.1%), followed by serum ferritin≥500μg/L (85.1%). Compared with the surviving group, children in the deceased group had lower WBC, lower HB, longer APTT, longer PT, lower FIB, higher central nervous system involvement, all with statistically significant differences (P<0.05). Central nervous system involvement and fibrinogen≤1.5g/L were independent prognostic factors of OS. Conclusions HLH is most often seen in infants, and the commonest clinical manifestation is fever with abnormally elevated serum LDH. Central nervous system involvement and FIB levels are important for the assessment of the outcome and prognosis of children with HLH.