Table 1

Diagnostic criteria for HLH

Fever 
Cytopenias in at least 2 cell lineages 
Hypertriglyceridemia and/or hypofibrinogenemia 
Hyperferritinemia 
High sCD25 (sIL-2R) concentration 
Splenomegaly 
Hemophagocytosis in different organs 
Low/absent NK cell cytotoxicity 
Fever 
Cytopenias in at least 2 cell lineages 
Hypertriglyceridemia and/or hypofibrinogenemia 
Hyperferritinemia 
High sCD25 (sIL-2R) concentration 
Splenomegaly 
Hemophagocytosis in different organs 
Low/absent NK cell cytotoxicity 
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