IL2RA and Splenomegaly: A modified HLH-2004 strategy has been proposed to assist with earlier diagnosis, which requires three of four clinical findings (fever, splenomegaly, cytopenias, and hepatitis), plus at least one of four immunologic test abnormalities (hyperferritinemia, elevated serum soluble IL-2Rα (sIL2R), absent/markedly decreased natural killer (NK) cell function, or the presence of hemophagocytosis) [39].