Malaria-associated secondary hemophagocytic lymphohistiocytosis: A case report

Table 2 Diagnosis of hemophagocytic lymphohistiocytosis-2004 criteria

The diagnosis of HLH can be established if either condition 1 or condition 2 below is fulfilled

(1) A molecular diagnosis consistent with HLH

(2) Diagnostic criteria for HLH fulfilled (five out of the eight criteria below)

(A) Initial diagnostic criteria (to be evaluated in all patients with HLH)

Fever

Splenomegaly

Cytopenia (affecting ≥ 2 of 3 lineages in the peripheral blood):

Hemoglobin < 90 g/L (in infants < 4 wk: hemoglobin < 100 g/L)

Platelets < 100 × 109/L

Neutrophils < 1.0 × 109/L

Hypertriglyceridemia and/or hypofibrinogenemia:

Fasting triglycerides ≥ 3.0 mmol/L (i.e., ≥ 265 mg/dL)

Fibrinogen ≤ 1.5 g/L

Hemophagocytosis in bone marrow or spleen or lymph nodes

No evidence of malignancy

(B) New diagnostic criteria

Low or absent NK-cell activity (according to local laboratory reference)

Ferritin ≥ 500 µg/L

Soluble CD25 (i.e., soluble IL-2 receptor) ≥ 2400 U/mL

HLH: Hemophagocytic lymphohistiocytosis.