Inherited TNFSF9 Deficiency Causes Broad Epstein–Barr Virus Infection With EBV+ Smooth Muscle Tumors

Scientists reported a patient with a heterozygous 22q11.2 deletion/DiGeorge syndrome who developed a unique, broad, and lethal susceptibility to Epstein–Barr virus (EBV) characterized by EBV-infected T and B cells and disseminated EBV+ smooth muscle cell tumors (SMT). They also demonstrated that wild-type CD137L was up-regulated on activated monocytes and dendritic cells, EBV-infected B cells, and SMT.