Several recent studies have identified germline homozygous or compound heterozygous mutations on Hepatitis A Virus-Cellular Receptor 2(HAVCR2) gene, encoding T-cell immunoglobulin and mucin domain-containing protein 3(TIM-3) in 59% of familial SPTCL (5),85% of sporadic Asian patients (6),and 25% of sporadic French cases (6),suggesting HAVCR2(TIM-3) germline mutations were susceptible to familial or sporadic SPTCL, and were associated with HLH/SPTCL. This evidence concerns the gene HAVCR2 and hemophagocytic syndrome.