Grant Awarded in 2017

Identifying differentiating biomarkers in an inception cohort of hyperferritinemic syndromes

Principal Investigator
Scott Canna
University of Pittsburgh
Pittsburgh, PA USA


Date of Award

December 2017
*Due to unforeseen circumstances, this project was canceled by the Investigator and funding was returned. 

Amount of Award
$34,155

Layperson Summary

Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS) are acute, life-threatening inflammatory histiocytic disorders that, despite advances in genetics and functional understanding, still result in significant organ damage and, too often, death. Importantly, most patients suspected of MAS or HLH are not classic for either, and often present a clinical challenge. The extent to which HLH and MAS are even distinct disorders is hotly debated, and in practice they are often treated very differently. Using samples obtained after patients were diagnosed, we found that the combination of biomarkers, IL-18 and CXCL9, could reliably distinguish classical forms of MAS from HLH. Importantly, both IL-18 and CXCL9 are associated with drugs either available or under development that could be used to intervene early in MAS and HLH.

If successful, this project may show us that patients can be divided early in their disease course between more “MAS-like” and more “HLH-like” based on these biomarkers. If this is the case, clinical trials taking advantage of this distinction will be organized that will hopefully show the efficacy of precisely distinguishing MAS from HLH and targeting the appropriate anti-inflammatory treatment before severe organ damage has had the chance to occur. This project will also establish a unique sample repository to explore future ideas related to early and precise diagnosis in these hemophagocytic syndromes.




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