45例噬血细胞综合征患儿的临床特点及预后分析

doi:10.12372/jcp.2025.25e0216

摘要/Abstract

摘要：

目的分析儿童噬血细胞综合征（HPS）的临床特点及预后影响因素。方法回顾性分析2017年1月至2023年7月收治的45例HPS患儿的临床资料及随访数据。结果 45例患儿中位发病年龄为4.5（2.1~10.0）岁，均以发热起病，感染相关HPS占77.8%，其中EB病毒（EBV）感染最多（85.7%）。所有患儿总生存率（88.9±4.7）%。单因素分析显示三酰甘油≥3.8 mmol/L、乳酸脱氢酶≥2 500 U/L是影响患儿预后的危险因素（P均<0.05），多因素分析显示三酰甘油≥3.8 mmol/L是影响患儿预后的独立危险因素（P=0.029）。结论 HPS常以发热起病，感染（尤其是EBV感染）为最常见的病因；初诊时三酰甘油及乳酸脱氢酶水平（尤其是三酰甘油≥3.8 mmol/L）是评估预后的关键因素。

关键词: 噬血细胞综合征, 临床特点, 治疗, 预后, 儿童

Abstract:

Objective To analyze the clinical characteristics and prognostic factors of hemophagocytic syndrome (HPS) in children. Methods A retrospective analysis was conducted on the clinical data and follow-up information of 45 children with HPS admitted from January 2017 to July 2023. Results The median age of onset in the 45 children was 4.5 (2.1-10.0) years, all of whom started with fever. Infection-related HPS accounted for 77.8%, with Epstein-Barr virus (EBV) infection being the most common (85.7%). The overall survival rate of all children was (88.9±4.7)%. Univariate analysis showed that triglycerides≥3.8 mmol/L and lactate dehydrogenase≥2500 U/L were risk factors affecting the prognosis of children with HPS (P<0.05 for both), and multivariate analysis showed that triglycerides≥3.8 mmol/L was an independent risk factor affecting the prognosis of children with HPS (P=0.029). Conclusion HPS often starts with fever, and infection (especially EBV infection) is the most common cause; the levels of triglycerides and lactate dehydrogenase at initial diagnosis (especially triglycerides≥3.8 mmol/L) are key factors in assessing prognosis.

Key words: hemophagocytic syndrome, clinical characteristics, treatment, prognosis, child

中图分类号:

蒋文轩, 叶芳华, 肖逸心, 杨良春. 45例噬血细胞综合征患儿的临床特点及预后分析[J]. 临床儿科杂志, 2025, 43(10): 762-767.

JIANG Wenxuan, YE Fanghua, XIAO Yixin, YANG Liangchun. Analysis of clinical characteristics and prognostic factors of 45 children with hemophagocytic syndrome[J]. Journal of Clinical Pediatrics, 2025, 43(10): 762-767.

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临床特征	累积生存率/%	χ²值	P
年龄		0.02	0.896
≤3岁	88.2±7.8
>3岁	89.3±5.8
sCD25		0.33	0.564
增高	90.6±5.2
正常	84.6±10.0
NK细胞活性^1）		0.13	0.716
降低	90.5±6.4
正常	87.0±7.0
血红蛋白		0.25	0.617
≤100 g·L^－1	87.5±5.8
>100 g·L^－1	92.3±7.4
白细胞计数		3.72	0.054
≤2.0×10⁹·L^－1	77.8±9.8
>2.0×10⁹·L^－1	96.3±3.6
中性粒细胞计数		2.68	0.101
≤0.5×10⁹·L^－1	80.0±8.9
>0.5×10⁹·L^－1	96.0±3.9
血小板计数		0.07	0.797
≤30×10⁹·L^－1	85.7±13.2
>30×10·L^－1	89.5±5.0
血清铁蛋白		0.00	0.966
≥10 000 ng·mL^－1	88.9±7.4
<10 000 ng·mL^－1	88.9±6.0
三酰甘油		10.65	0.001
≥3.8 mmol·L^－1	63.6±14.5
<3.8 mmo·L^－1	97.1±2.9
纤维蛋白原		0.02	0.899
≤1.5 g·L^－1	88.5±6.3
>1.5 g·L^－1	89.5±7.0
乳酸脱氢酶		5.43	0.020
>2 500 U·L^－1	73.3±11.4
≤2 500 ·L^－1	96.7±3.3
丙氨酸氨基转移酶		0.38	0.540
升高	88.1±5.0
正常	100
天门冬氨酸氨基转移酶		0.97	0.324
升高	86.8±5.5
正常	100
总胆红素		0.21	0.648
升高	92.3±7.4
正常	87.5±5.8
直接胆红素		0.31	0.576
升高	92.9±6.9
正常	87.1±6.0
白蛋白		0.31	0.581
≤30 g·L^－1	84.6±10.0
>30 g·L^－1	90.6±5.2

影响因素	β	S.E.	Wald χ²	P	HR（95%CI）
白细胞计数≤2.0×10⁹·L^－1	0.844	1.198	0.496	0.481	2.325（0.222~24.322）
三酰甘油≥3.8 mmol·L^－1	2.592	1.191	4.739	0.029	13.360（1.295~137.862）
乳酸脱氢酶>2 500 U·L^－1	2.172	1.143	3.611	0.057	8.772（0.934~82.381）