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Table 2 Diagnostic criteria for HLH*

From: Macrophage activation syndrome in a patient with pulmonary inflammatory myofibroblastic tumour

(1) Fever

Manifestations in the patient described quotidian fever over several weeks

(2) Splenomegaly

hepatosplenomegaly

(3) Cytopenia involving two or more cell lines

haemoglobin < 9.0 d/dL, no other

(4) Hypertriglyceridaemia or hypofibrinogenaemia

no

(5) Haemophagocytosis

yes

(6) Hepatitis

yes

(7) Low or absent natural killer cell activity

yes

(8) Serum ferritin level > 500 μg

1043 μg/L

(9) Soluble CD25 (sIL-2 receptor) > 2500 U/ml

> 7200 U/ml

  1. *according to HLH-2004 criteria (Henter JI et al.).