In humans, germline autosomal recessive TIM-3 deficiency (p.Tyr82Cys, p.Ile97Met) predisposes to rare subcutaneous panniculitis-like T cell lymphoma (SPTCL) complicated by hemophagocytic lymphohistiocytosis (HLH) [5]. This evidence concerns the gene HAVCR2 and hemophagocytic syndrome.