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Table 3 Hemophagocytic lymphohistiocytosis (HLH) criteria[16]

From: Algorithm development for corticosteroid management in systemic juvenile idiopathic arthritis trial using consensus methodology

Criteria (5 or more of the following)
Fever (last 4/7 days) Hemophagocytosis in tissue
Splenomegaly TG ≥265 mg/dl
Bi-cytopenia (affecting ≥ 2 of 3 cell lineages) Platelets < 100,000/ml Neutrophils <1000/ml, Hgb < 9 g/dl Ferritin ≥500 mcg/L
Low NK (natural killer cells) activity Elevated CD25 (sIL-2R) ≥ 2,400 U/ml
Fibrinogen ≤ 1.5 g/L