Moleculobiological pathogenesis, diagnosis and treatment of epstein-barr virus-associated hemophagocytic lymphohistiocytosis in children
10.3760/cma.j.issn.1673-4408.2016.06.002
- VernacularTitle: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
- Author:
Guiying ZHU
- Publication Type:Journal Article
- Keywords:
Hemophagocytic lymphohistiocytosis;
Epstein-barr virus;
Moleculobiological pathogenesis;
Diagnosis;
Treatment
- From:
International Journal of Pediatrics
2016;43(6):430-433
- CountryChina
- Language:Chinese
-
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.
