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Frontiers in immunology, Vol. 12, Pages 612583, 2021
PMID: 33746956
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of immune dysregulation characterized by hyperactivation of the immune system, excessive cytokine secretion and severe systemic inflammation. HLH is classified as familial (FHL) when associated with mutations in PRF1, UNC13D, STX11, and STXBP2 genes.
https://www.ncbi.nlm.nih.gov/pubmed/33746956
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