Moleculobiological pathogenesis, diagnosis and treatment of epstein-barr virus-associated hemophagocytic lymphohistiocytosis in children / 国际儿科学杂志

ABSTRACT

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.