Abstract
Hemophagocytic lymphohistiocytosis (HLH), which was first described in 1939 by paediatricians Scott and Robb-Smith, is a life-threatening disease. HLH is characterized as cytokine release syndrome which is caused by excessive but non-malignant activation of macrophages and/or histiocytes in bone marrow and other reticuloendothelial systems. EBV-HLH is the most common type of infection-associated HLH, has a high mortality rate without prompt and effective treatment. A previous study showed that the one-year mortality rate of EBV-HLH patients is 75%. Here we report a case of EBV-associated hemophagocytic syndrome in adult, and the lessons from the treatment process. Through this case, we think that for EBV-related HLH, EBV-DNA should also be monitored in addition to hemophagocytosis-related indicators during treatment. In addition, DEP regimen may not be suitable for patients who have received at least partial response, because impaired immunological functioning may lead to EBV and hemophagocytic re-activity.
Highlights
DEP regimen may not be suitable for patients who have received at least partial response, because impaired immunological functioning may lead to Epstein-Barr virus (EBV) and hemophagocytic re-activity
HLH is divided into two categories: primary and secondary. The former mainly occurs in children, most of whom have a family genetic history, and is accompanied by genetic abnormalities, while the latter can occur at all ages, mostly caused by infections, tumors and autoimmune diseases [2]
We found that antiviral therapy may be an effective treatment for EBV-related hemophagocytic syndrome (HPS), especially with high viral load of EBV-DNA and negative results of EBV-infected lymphocyte subsets
Summary
Wanqiu Zhang*, Jiakui Zhang*, Qianshan Tao, Qing Zhang, Yinwei Li, Fan Wu, Zhimin Zhai*#.
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