儿童噬血细胞综合征28例临床特点及预后分析

Clinical features and prognosis of 28 children with hemophagocytic syndrome

目的分析噬血细胞综合征(HPS)患儿的临床特点和预后因素。方法回顾性分析28例HPS患儿的临床表现及相关实验室指标,采用Logistic多因素回归分析与预后相关的危险因素。结果 HPS患儿以持续高热(100%)、肝肿大(64.29%)为突出表现,其次表现为呼吸系统症状(53.57%)、脾肿大(50.00%)、多浆膜腔积液(42.86%)、淋巴结肿大(32.14%)、黄疸(17.86%)、中枢神经系统症状(14.29%)、皮疹(14.29%)和消化道出血(10.71%)。实验室检查以肝功能损害、全血细胞减少、凝血功能障碍、高三酰甘油血症、自然杀伤细胞数量降低及低钠血症等为主。病因分析显示,感染相关性HPS 17例(60.71%),其中以EB病毒相关性HPS最多(11例,感染相关性HPS的64.71%)。死亡与存活病例间的血清白蛋白、尿素氮和活化部分凝血活酶时间差异有统计学意义(P<0.05)。Logistic多因素分析显示,血清白蛋白<35 g/L是HPS患儿死亡的独立危险因素(OR=41.33,P=0.026)。结论 HPS病因复杂,临床表现多样,病情进展迅速,血清白蛋白水平是影响HPS预后的独立危险因素,及时确诊并干预治疗对预后至关重要。

Objectives To investigate the clinical profile and prognosis of hemophagocytic syndrome （HPS）. Methods A retrospective study was carried out to analyze the clinical features and laboratory findings in 28 children with HPS. Fisher＇s exact probability method and Logistic multivariate regression were used to explore the prognostic risk factors.. Results HPS was clinically characterized by prolonged fever （100%）, hepatomegaly （64.29%）,and other minor features including respiratory symptoms （53.57%）, splenomegaly （50%）, hydrops of multiple serous cavity （42.86%）, lymphadenectasis （32.14%）, jaundice （17.85%）, skin rash （14.29%）, central nervous system involvement （14.29%）, and alimentary tract hemorrhage （10.71%）. Labo-ratory data showed that 1iver dysfunction, pancytopenia, coagulation abnormalities, disseminated intravascular coagulation, hy-pertriglyceridemia, decreased number of natural killer cells and hyponatremia were prominent. The etiological analysis indicated that infection associated hemophagocytic syndrome was most common （60.71%）, in which EB virus associated HPS was pre-dominant, accounting for 64.71%. Significant difference was observed in the difference of albumin,blood urea nitrogen and acti-vated partial thromboplastin time between death and survival cases （P〈0.05）. The Logistic regression multivariate analysis showed that hypoalbuminemia was an independent prognostic factor. Conclusions There are various underlying diseases and clinical manifestations for HPS. The lower level of serum albumin is an independent prognostic factor. A prompt diagnosis and treatment is very important for HPS prognosis due to the rapid progression and high mortality.