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Table 1 Diagnostic criteria for HLH [4]

From: Hemophagocytic lymphohistiocytosis complicating a T-cell rich B-cell lymphoma

≥ five of the eight criteria listed below:

 

Fever

 

Splenomegaly

 

 Cytopenias (affecting at least two of three lineages in the peripheral blood):

 • Hemoglobin < 9 g/dl

 • Platelets < 100 G/mm3

 • Neutrophils <1 G/mm3

 

Hypertriglyceridemia (fasting, 265 mg/100 ml) and/or hypofibrinogenemia (150 mg/100 ml)

 

Hemophagocytosis in bone marrow, spleen or lymph nodes

 

Ferritin ≥ 500 ng/ml

 

Low or absent Natural Killer cell activity

 

Soluble IL-2 receptor ≥ 2400 U/ml