Table 2 Diagnosis of HLH
One of the two conditions is met | |
|---|---|
(a) Pathological mutations are found in the HLH-related pathogenic genes. | |
(b) At least five of the following eight criteria are met. | |
1. Fever: temperature > 38.5℃. | |
2. Hemocytopenia: hemoglobin < 90 g/L, platelets < 100*109/L, neutrophils < 1.0*109/L and not due to bone marrow hypoplasia. | |
3. Splenomegaly. | |
4. Hypertriglyceridemia > 3mmol/L or hypofibrinogenemia < 1.5 g/L. | |
5. Phagocytes found in bone marrow, spleen or lymph nodes. | |
6. Serum ferritin ≥ 500 µg/L. | |
7. Decreased or absent NK cell activity. | |
8. Elevated soluble interleukin-2 receptor (sCD25) level ≥ 2400µ/ml. |