儿童继发性噬血细胞综合征的临床特征及预后分析  

Clinical characteristics and prognosis of secondary hemophagocytic lymphohistiocytosis in children

作　　者：常莉[1] 崔亚利[1] 彭磊文[1] 刘婷[1] 刘芳[1] CHANG Li;CUI Ya-li;PENG Lei-wen;LIU Ting;LIU Fang(Department of Laboratory Medicine, West China Second Hospital, Sichuan Universit;Key Laboratory of Birth Defect and Related Maternal and Child Diseases of Ministry of Education, Chengdu 610041, Chin)

机构地区：[1]四川大学华西第二医院检验科/出生缺陷与相关妇儿疾病教育部重点实验室,成都610041

出　　处：《解放军医学杂志》2018年第5期440-445,共6页Medical Journal of Chinese People's Liberation Army

基　　金：四川省科技厅重点研发项目(2017SZ0122)~~

摘　　要：目的探讨儿童继发性噬血细胞综合征(HLH)的病因、临床特征及预后。方法回顾性分析四川大学华西第二医院84例继发性HLH患儿的病因、临床特征、实验室指标及预后结局。结果 84例患儿中,63例(75.0%)为感染性,其中EB病毒(EBV)感染45例,11例(13.1%)为免疫性,10例(11.9%)为血液肿瘤性。临床主要表现为持续发热(100%)、肝肿大(83.3%)、脾肿大(67.9%)、淋巴结肿大(59.5%),其次为皮肤瘀斑(26.2%)、皮疹(19.0%)、消化道症状(15.5%)及神经系统症状(9.5%)。实验室检查以外周血两系及以上减少、凝血功能障碍、肝酶升高、高甘油三酯血症、血清铁蛋白升高及NK细胞数量减少为主,73.8%的患儿骨髓查见噬血现象。84例患儿中放弃治疗4例,好转58例,死亡16例,失访6例,3年累积生存率为77.2%±5.1%,不同病因组间累积生存率差异无统计学意义(P>0.05)。死亡与存活患儿的年龄、中枢神经系统累及率、血小板(PLT)、凝血酶原时间(PT)、活化部分凝血酶原时间(APTT)、纤维蛋白原(Fg)、乳酸脱氢酶(LDH)、白蛋白(ALB)差异有统计学意义(P<0.05)。Logistic多因素回归结果显示LDH>2000U/L及ALB<30g/L是HLH死亡的独立预后因素(P<0.05)。结论儿童继发性HLH由多种病因所致,以感染为主;临床表现多样,进展迅速,血清白蛋白和LDH水平是影响其预后的危险因素,应尽早诊断,及时治疗。Objective To investigate the etiology, clinical characteristics and prognosis of secondary hemophagocytic lymphohistiocytosis（HLH） in children. Methods The clinical data, collected in the West China Second Hospital of Sichuan University, were retrospectively reviewed of etiology, clinical characteristics, laboratory results and prognosis of 84 children with secondary HLH. Results Of the 84 cases, 63 cases（75.0%） were with infections, especially 45 cases were EBV infection, 11 cases（13.1%） were with immune disorder and 10 cases（11.9%） with malignancies. HLH was clinically characterized by prolonged fever（100%）, hepatomegaly（83.3%）, splenomegaly（67.9%）, lymphadenectasis（59.5%）, and other minor features including ecchymosis（26.2%）, rash（19.0%）, alimentary tract（15.5%） and central nervous system involvement（9.5%）. Laboratory data showed that pancytopenia, coagulation abnormalities, liver dysfunction, hypertriglyceridemia, elevated serum ferritin and decreased number of NK cells were prominent, and hemophagocytosis were found in bone marrow of 73.8% cases. Four of 84 cases gave up treatment, improvement in 58 cases, 16 cases died and 6 cases lost of follow-up. The 3 year cumulative survival rate was 77.2%±5.1%, and no significant difference existed on cumulative survival rates among the different etiological groups（P〈0.05）. Significant differences（P〈0.05） were observed between survived and died cases on age, central nervous system implication, platelets（PLT）, prothrombin time（PT）, activation prothrombin time（APTT）, fibrinogen（Fg）, lactate dehydrogenase（LDH） and albumin（ALB）. Logistic multivariate regression showed that LDH 2000 U/L and ALB30 g/L were the independent prognostic factors led to death of HLH cases. Conclusions Children secondary HLH has multiple etiologies and diversified clinical features. The serum levels of LDH and ALB are the independent prognostic factors. Early diagnosis and timely treatment are very important for H

关 键 词：噬血细胞综合征 继发性 儿童 临床特征 预后 

分 类 号：R725.4[医药卫生—儿科]