摘要
目的探讨儿童噬血细胞综合征(HPS)的临床表现、病因、治疗及预后。方法回顾性分析2004年1月-2006年12月青岛大学医学院附属医院儿科收治的22例HPS患儿临床资料,总结其特点。结果1.临床表现:主要为持续发热,肝、脾、淋巴结大,出血。2.实验室检查特点:外周血常规表现为三系或二系减少,肝功能受损,凝血障碍,高三酰甘油和(或)纤维蛋白原降低。骨髓涂片可找到噬血细胞。免疫紊乱:CD3、CD4降低,CD8升高。3.病因:原发性和获得性,获得性包括感染和恶性肿瘤相关,其中以EB病毒最为常见,EB病毒感染占感染相关性HPS(IAHS)的63.64%(7/11例),占HPS的31.82%(7/22例)。EBV相关HPS表现重,尤其EBV DNA定量阳性者临床凶险,病死率高。1例恶性相关性HPS(MAHS)有EBV DNA低量表达,且EBV-CA-IgM阳性,为EB病毒感染,余3例病因不明。4.治疗及转归:IAHS 11例予抗病毒药物、大剂量人血丙种球蛋白、糖皮质激素、依托泊苷治疗,好转5例,疗效不佳自动出院1例,放弃治疗2例,死亡3例。MAHS 4例,予糖皮质激素、依托泊苷等治疗,对早期治疗反应良好,易复发,复发后恶性病程,预后差。1例0.5 a余转化为淋巴瘤;1例10个月余转为急性淋巴细胞性白血病;1例3个月余转为郎格汉斯组织细胞增生症,进行性急速加重;前3例均迅速死亡。1例EBV感染MAHS,最终转化为郎格汉斯组织细胞增生症,肝功能不良,持续3 a。家族性噬血细胞淋巴组织细胞增生症1例,经HLH-04方案治疗好转,近1 a复发,死于多脏器衰竭,中枢浸润。6例病原不清HPS,亦予上述治疗,死亡、好转各3例。结论HPS多由感染尤其为EBV感染诱发,且与细胞免疫有极大关系,早期诊断、早期治疗是改善预后的关键。
Objective To explore the clinical features,etiological factors,treatments and prognosis factors in children with hemo-phagocytic syndrome（HPS）.Methods Twenty-two children with HPS from Jan.2004 to Dec.2006 were retrospectively analyzed,their clinical features,etiological factors,treatments and prognosis factors were reviewed.Results 1.Main clinical features：persistent fever,hepatosplenomegaly,lymphadenopathy,hemorrage.2.Characteristic laboratory findings：cytopenia≥2 cell lines,abnormal liver function and coagulation function,hypertriglyceridema and（or） decreased fibrinogen,hemophagocytosis in bone marrow,and immunologic disturbances：decreased both CD3 and CD4,elevated CD8.3.Etiological factors：genetic and acquired HLH.Acquired HLH includes infection and malignancy associa-ted HLH.EBV was the major triggering virus：7（63.64%,31.82%）cases in infection associated hemophagocytic syndrome（IAHS） and malignancy associated hemophagocytic syndrome（MAHS）,respectively.EBV associated HLH were serious,especially cases with positive EBV DNA had fulminant course and high mortality.One of MAHS was both EBV DNA positive and EBV-CA-IgM positive.4.Treatments and effects：11 cases of IAHS received antivirus,high dose of immunoglobin,glucocorticoid and etoposide treatment,5 cases improved,1 case discharged automaticly because of bad effect,2 cases refused to receive further treatment,3 cases died.Four cases of MAHS received glucocorticoid,etoposide treatment,1 case transformed to lymphoma in half a year;one case to leukemia in 10 months;one case to Langerhans cell his-tiocytosis（LCH） in 3 months;4 cases of MAHS had good therapeutic reaction at early stages of treatment,but once relapsed,they all had malignant courses,poor prognosis and died rapidly.One of them with EBV infection to LCH had persistent abnormal liver function,lasting for 3 years.One case of familial hemophagocytic lymphohistiocytosis were treated with HLH-04 protocol,relapsed after 1 year,died of multiorgan failure with central nervous system involvement.Six cases of HLH with unidentified etiologic causes received the same treatment,3 cases died and 3 cases improved.Conclusions HPH was triggered by infections especial EBV infection,and was greatly related to cell immunity;early diagnosis and early treatment are the key to improve prognosis.
出处
《实用儿科临床杂志》
CAS
CSCD
北大核心
2008年第15期1183-1186,共4页
Journal of Applied Clinical Pediatrics
关键词
噬血细胞综合征
病因
细胞免疫
hemophagocytic syndrome
etiology
cell immunity
