An example is loss-of-function mutations involving the Hepatitis A Virus-Cellular Receptor 2 gene (HAVCR2), which are enriched in SPTCL and result in dysregulated interferon-γ-inducible inflammatory host response as predisposing factor for HLH [155–157]. Here, HAVCR2 is linked to subcutaneous panniculitis-like T-cell lymphoma.