Case Report
Copyright ©The Author(s) 2021.
World J Clin Cases. Aug 6, 2021; 9(22): 6403-6409
Published online Aug 6, 2021. doi: 10.12998/wjcc.v9.i22.6403
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