Objective: To explore the clinical characteristics, prognostic risk factors and effective treatment of severe hemophagocytic syndrome (HPS) in children, so as to provide reference for the clinical diagnosis and treatment of the disease. Methods: The clinical data of 83 children with severe HPS admitted in Affiliated Hospital of Zunyi Medical University from January 2014 to April 2021 were collected, and their clinical characteristics, prognosis and prognostic risk factors were analyzed. The children were divided into central nervous system (CNS) dysfunction group and non-CNS dysfunction group according to whether they were accompanied with CNS dysfunction, and were divided into blood purification group and non-blood purification group according to whether they received blood purification, then the survival differences were compared. Results: Among the 83 children, there were 43 males and 40 females, aged[M(Q1,Q3)] 36(15,27)months. A total of 51 children were induced by infection, among which 41 children (80.4%) were infected with EB virus. All the children were accompanied by multiple organ dysfunction (MODS), and dysfunction of the blood system (72.3%), liver (71.1%), respiratory system (53.0%) and CNS (37.3%) were common. By the end of follow-up, 40 cases (48.2%) survived, 38 cases (45.8%) died, and 5 cases (6.0%) were lost to follow-up. CNS dysfunction was a risk factor (HR=3.358, 95%CI: 1.445-7.803, P=0.005) and blood purification was a protective factor (HR=0.362, 95%CI: 0.179-0.730, P=0.005) affecting the prognosis of children. The mortality of CNS dysfunction group was statistically higher than that of non-CNS dysfunction group (74.2% vs 28.8%) (P<0.001); The mortality of blood purification group was statistically lower than that of non-blood purification group (31.0% vs 61.0%) (P=0.010). Conclusions: Severe HPS in children was dangerous and had a poor overall prognosis. CNS dysfunction was a risk factor for death. Blood purification could significantly improve the prognosis and improve the survival rate of children.
目的： 探讨儿童重型噬血细胞综合征（HPS）的临床特征、预后危险因素及有效治疗方法，为该病的临床诊治提供参考。 方法： 收集2014年1月至2021年4月遵义医科大学附属医院收治的83例重型HPS患儿的临床资料，分析其临床特征、转归及预后危险因素。根据合并中枢神经系统（CNS）功能障碍与否将患儿分为CNS功能障碍组及非CNS功能障碍组，根据接受血液净化与否将患儿分为血液净化组及非血液净化组，比较生存差异。 结果： 83例患儿中，男43例，女40例，年龄［M（Q1，Q3）］36（15，27）个月。51例由感染诱发，其中EB病毒感染41例（80.4%）。所有患儿均伴多器官功能障碍（MODS），以血液系统（72.3%）、肝脏（71.1%）、呼吸系统（53.0%）及CNS（37.3%）功能障碍常见。至随访截止，存活40例（48.2%），死亡38例（45.8%），失访5例（6.0%）。CNS功能障碍是影响患儿预后的危险因素（HR=3.358，95%CI：1.445~7.803，P=0.005），血液净化治疗是影响患儿预后的保护因素（HR=0.362，95%CI：0.179~0.730，P=0.005）。CNS功能障碍组死亡率高于非CNS功能障碍组（74.2%比28.8%），两组差异有统计学意义（P<0.001）；血液净化组死亡率低于非血液净化组（31.0%比61.0%），两组差异有统计学意义（P=0.010）。 结论： 儿童重型HPS病情凶险，总体预后差，CNS功能障碍是其死亡的危险因素，血液净化可以显著改善其预后，提高患儿生存率。.