What are HLH-2004 criteria?

By William Aird

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.
  • The HLH-2004 criteria, developed for children, are not validated formally for adults and remain based on expert opinion.

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