Diagnostic criteria for HLH — established by a molecular diagnosis or by 5 of 8 clinical/laboratory findings.

The diagnosis of HLH is established by fulfilling one of the following 2 criteria:

1) A molecular diagnosis consistent with HLH (e.g., PRF mutations, SAP mutations)

2) Presence of 5 of the following 8 symptoms, signs, or laboratory abnormalities:

a) Fever
b) Splenomegaly
c) Cytopenia (affecting ≥2 cell lineages; hemoglobin ≤9 g/dL [or ≤10 g/dL for infants <4 wk old], platelets <100,000/µL, neutrophils <1,000/µL)
d) Hypertriglyceridemia (≥265 mg/dL) and/or hypofibrinogenemia (≤150 mg/dL)
e) Hemophagocytosis in the bone marrow, spleen, or lymph nodes without evidence of malignancy
f) Low or absent natural killer cell cytotoxicity
g) Hyperferritinemia (≥500 ng/mL)
h) Elevated soluble CD25 (interleukin-2Rα chain; ≥2,400 U/mL)

Source: Adapted from Verbsky JW, Grossman WJ: Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives, Ann Med 38:20–31, 2006 (Table 1, p 21).

Hemophagocytic Lymphohistiocytosis

The diagnosis of HLH is established by fulfilling one of the following 2 criteria:

2) Presence of 5 of the following 8 symptoms, signs, or laboratory abnormalities:

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