Progress in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis(HLH)is a severe inflammatory syndrome caused by the exces-sive proliferation and activation of T cells and macrophages.This disease progresses rapidly with a high mortality rate.Although the HLH-1994 and HLH-2004 protocols have significantly improved patient survival rates,there are still therapeutic challenges.Epstein-Barr virus-associated HLH(EBV-HLH)is the most common subtype of HLH in Asian children.Although the outcome of pediatric EBV-HLH is superior to other subtypes,about 20%~30%of patients experience relapse or arerefractory to the treatment.This review provides an overview of the ad-vancements in the treatment for EBV-HLH.