Treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis: Study protocol of a prospective pilot study

Figure 2 Correlations between hemophagocytic lymphohistiocytosis and chronic active Epstein-Barr virus infection status. CAEBV status may occur without apparent onset of symptoms or may develop following initial acute onset EBV-HLH. During the course of CAEBV, HLH episodes may develop, and if it is not adequately treated by transplantation, most patients eventually succumb to terminal HLH or to lymphoid malignancies. CD8+ T cells play a major role in initial acute onset HLH, whereas CD4⁺ T cells or NK cells play a role in the status of CAEBV and in CAEBV-related HLH. CAEBV: Chronic active EBV infection; EBV-HLH: Epstein-Barr virus-related hemophagocytic lymphohistiocytosis; NK: Natural killer.