Seven years later, the 10-year-old girl developed a prolonged fever and fatigue, and she was diagnosed as presenting CAEBV and EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) due to the presence of a high EBV-DNA load, prolonged fever, splenomegaly, bicytopenia, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis, low NK-cell activity, and increased levels of ferritin and soluble CD25. Here, IL2RA is linked to Splenomegaly.