HLH- 2004 diagnostic criteria. Diagnosis of HLH can be established if Criterion 1 or 2 is fulfilled [14].

Table 4: HLH- 2004 diagnostic criteria.
Diagnosis of HLH can be established if Criterion 1 or 2 is fulfilled [14].

HLH-2004 Diagnostic Criteria	Criteria satisfied by patient
1. A molecular diagnosis consistent with HLH	Yes
2. Dx criteria for HLH (5 of 8 required)	-
Fever	Yes
Splenomegaly	Yes
Cytopenias (affecting at least 2 of 3 lineages)	-
Hemoglobin <90 g/L	-
Platelets <100 ×10^9 /L	-
Neutrophils <1.0 ×10^9/L	-
Hypertriglyceridemia (>265 ng/dL) and/or Hypofibrinogenemia (<1.5g/L)	-
Hemophagocytosis in the bone marrow, spleen or lymph node	-
Low to no NK cell activity	-
Ferritin >500 ug/L	Yes
sCD25 > 2400 U/mL	-