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
In this manuscript, a number of debatable issues related to the diagnosis and treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis (EBV-HLH) will be addressed. Considering the heterogeneous nature of EBV-HLH, diagnostic efforts are required to clarify the precise nature of the disease at diagnosis, the number of EBV genome copies in peripheral blood, and localization of the EBV genome in lymphoid cells (B, T, or natural killer cells). Although the majority of cases of EBV-HLH develop without evidence of immunodeficiency, some cases have been found to be associated with chronic active EBV infection, genetic diseases such as X-linked lymphoproliferative disease (XLP, type 1, or type 2), or familial HLH (FHL, types 2-5). Due to such background heterogeneity, the therapeutic results of EBV-HLH have also been found to vary. Patients have been found to respond to corticosteroids alone or an etoposide-containing regimen, whereas other patients require hematopoietic stem cell transplantation. Thus, decision-making for optimal treatment of EBV-HLH and its eventual outcome requires evaluation in consideration of the precise nature of the disease. A protocol for a pilot study on the treatment of patients with EBV-HLH is presented here.
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.