Diagnostic Guidelines for HLH
| Fever: duration ≥7 days with peaks ≥38.5°C |
| Splenomegaly: 3 cm or more below the costal margin |
| Two of the following hematological abnormalities: |
| Anemia: hemoglobin <90 g/L |
| Thrombocytopenia: platelets <100 × 109/L |
| Neutropenia: neutrophils <1.0 × 109/L |
| Hypertriglyceridemia: ≥2.0 mmol/L or 3 SD over the normal value for the patients age and/or Hypofibrinogenemia: fibrinogen ≤1.5 g/L or 3 SD below the normal value for the patients age |
| Hemophagocytosis in bone marrow, spleen, or lymph node. No evidence of malignancy. |
| HLH: all of the above criteria are required for the diagnosis of HLH. |
| Familial HLH: Diagnosis of FHL is justified in the presence of a family history of HLH and all criteria listed above. |
| Fever: duration ≥7 days with peaks ≥38.5°C |
| Splenomegaly: 3 cm or more below the costal margin |
| Two of the following hematological abnormalities: |
| Anemia: hemoglobin <90 g/L |
| Thrombocytopenia: platelets <100 × 109/L |
| Neutropenia: neutrophils <1.0 × 109/L |
| Hypertriglyceridemia: ≥2.0 mmol/L or 3 SD over the normal value for the patients age and/or Hypofibrinogenemia: fibrinogen ≤1.5 g/L or 3 SD below the normal value for the patients age |
| Hemophagocytosis in bone marrow, spleen, or lymph node. No evidence of malignancy. |
| HLH: all of the above criteria are required for the diagnosis of HLH. |
| Familial HLH: Diagnosis of FHL is justified in the presence of a family history of HLH and all criteria listed above. |
Abbreviation: FHL, familial hemophagocytic lymphohistiocytosis.