Hemophagocytic lymphohistiocytosis (HLH), which was first described in\n1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease.\nHLH is characterized as cytokine release syndrome which is caused by excessive\nbut non-malignant activation of macrophages and/or histiocytes in bone\nmarrow and other reticuloendothelial systems. EBV-HLH is the most common\ntype of infection-associated HLH, has a high mortality rate without\nprompt and effective treatment. A previous study showed that the one-year\nmortality rate of EBV-HLH patients is 75%. Here we report a case of\nEBV-associated hemophagocytic syndrome in adult, and the lessons from the\ntreatment process. Through this case, we think that for EBV-related HLH,\nEBV-DNA should also be monitored in addition to hemophagocytosis-\nrelated indicators during treatment. In addition, DEP regimen may not be\nsuitable for patients who have received at least partial response, because impaired\nimmunological functioning may lead to EBV and hemophagocytic\nre-activity.
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