Hemophagocytic lymphohistiocytosis (HLH) and Macrophage activation syndrome (MAS) are complex hyperinflammatory syndromes, which are difficult to distinguish from issues such as sepsis, as the root cause (high level proinflammatory cytokine production) is very similar. This causes confusion among treating physicians, and diagnostic/treatment delays. Furthermore, in adult patients, HLH is often associated with hematolymphoid malignancies, and a thorough search for these should be undertaken in patients in whom HLH is suspected or diagnosed, so as to best direct treatment. The treatment of HLH in children (in whom genetic defects in T cell function often underlie the disease) is standardized and protocol driven. In adults it is currently quite variable, in part due to the variability in underlying causes. The delays in diagnosis, the frequent associations with malignancy and the variability in treatment approaches, all contribute to poorer mortality in adult HLH patients. Recognizing the need for a consensus, multidisciplinary approach to these complicated patients, and the need for research to better their care, we have created a multidisciplinary working group, which is accessible to all physicians at UT Southwestern and Parkland, to facilitate this.

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