Hemophagocytic lymphohistiocytosis: Recent progress in the pathogenesis, diagnosis and treatment

Table 2 Diagnostic guidelines for hemophagocytic lymphohistiocytosis[38]

The diagnosis of HLH can be established if one of either (1) or (2) below is fulfilled
(1) A molecular diagnosis consistent with HLH
(2) Clinical diagnostic criteria fulfilled for 5 out of the 8 criteria below
Clinical criteria	1 fever
	2 splenomegaly
Routine laboratory criteria	3 bicytopenia (Hb < 90 g/L,
	platelets < 100 × 109/L, neutrophils < 1.0 × 109/L)
	4 Hypertriglyceridemia (> 3.0 mmol/L)
	and/or hypofibrinogenemia (< 1.5 g/L)
Specific histopathological/marker criteria	5 hemophagocytosis
	6 low or absent NK cell activity
	7 hyperferritinemia (> 500 μg/L)
	8 hyper-sIL-2R-nemia (> 2400 U/mL)

HLH: Hemophagocytic lymphohistiocytosis; NK: Natural killer.