TIM3 deficiency (OMIM #618398) caused by HAVCR2 mutations is another autosomal-recessive inborn error of immunity that predisposes to HLH in a particular context, i.e., in subcutaneous panniculitis T cell lymphoma (SPTCL) (30). Here, HAVCR2 is linked to subcutaneous panniculitis-like T-cell lymphoma.