Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome, its diagnosis being based on fulfilment of five out of eight clinical and laboratory parameters (fever, splenomegaly, cytopenia, hemophagocytosis, ferritin>500 ng/ml, hypertrygliceridemia and/or hypofibrinogenemia, low or absent NK cell activity, increased of soluble receptor for IL2) or a molecular diagnosis of a disease conferring a high risk of developing HLH
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