To elucidate the pathogenesis of SPTCL/HLH clinical spectrum, germline mutations in the hepatitis A virus cellular receptor (HAVCR2) gene have been discovered to alter the T-cell immunoglobin and mucin-domain containing-3 (TIM-3) protein leading to immune dysregulation and amplified inflammatory signaling [5, 6]. Here, HAVCR2 is linked to hemophagocytic syndrome.