What are the Histiocyte Society HLH-2004 criteria for diagnosis of hemophagocytic lymphohistiocytosis (HLH)?
Clinical criteria for predicting the diagnosis of hemophagocytic lymphohistiocytosis (HLH):
- In 1991, the Histiocyte Society proposed a standardized set of 5 diagnostic criteria for HLH used for the prospective HLH-94 clinical trial.
- These criteria were revised for HLH-2004: individuals needed to meet 5 or more of 8 diagnostic criteria.
- Hypertriglyceridemia or hypofibrinogenemia
- Hemophagocytosis in bone marrow, spleen, or lymph nodes
- Low or absent NK-cell activity
- Ferritin ≥ 500 mcg/L
- Elevated soluble interleukin-2 receptor.
- The HLH-2004 criteria, developed for children, are not validated formally for adults and remain based on expert opinion.
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