Published online Nov 6, 2015. doi: 10.5315/wjh.v4.i4.69
Peer-review started: June 4, 2015
First decision: August 14, 2015
Revised: September 6, 2015
Accepted: October 12, 2015
Article in press: October 13, 2015
Published online: November 6, 2015
Core tip: Diagnosis of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH) must fulfill both the evidence of EBV infection and the diagnostic criteria for HLH. EBV-HLH is heterogeneous. The majority of EBV-HLH occurs in apparently immunocompetent subjects, but some are associated with chronic active EBV infection status, X-linked lymphoproliferative disease or with familial HLH. Thus, treatment and outcome differ significantly depending on the underlying disease. To find out a most appropriate treatment, various laboratory tests are required to clarify the underlying diseases.