Table 2.

Diagnostic criteria for HLH*

(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

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