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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