儿童EB病毒相关噬血细胞综合征的分子生物学机制和诊疗研究进展

EB病毒相关噬血细胞综合征起病凶险,进展迅速,预后差,未经治疗者病死率高.其具体发病机制目前尚不完全清楚,可能与SH2D1A基因突变和EBV潜在膜蛋白1有关.诊断首先需符合噬血细胞性淋巴组织细胞增多症诊断标准,并存在EB病毒感染证据,同时排除原发性噬血.治疗上首选联合化疗,若化疗效果不佳,可行造血干细胞移植和单克隆抗体治疗.

Moleculobiological pathogenesis,diagnosis and treatment of epstein-barr virus-associated hemophagocytic lymphohistiocytosis in children

Epstein-Barr (EB) virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) onset dangerous,rapid progress,the prognosis is poor,with high mortality without treatment.The specific pathogenesis is not entirely clear,it may be related to SH2D1A gene mutation and EBV potential membrane protein 1 (LMP1) concerned.Diagnosis first need to comply with hemophagocytic lymphohistiocytosis (HLH) diagnostic criteria,and there is evidence of EB virus infection,while excluding primary HLH.The preferred treatment in combination with chemotherapy,if the poor effect of chemotherapy,viable hematopoietic stem cell transplantation and monoclonal antibody therapy.