Neves, JFRaga, LTChiang, SCCTesi, BVieira, JPCordeiro, AIBorrego, LBryceson, YT2021-04-292021-04-292019Pediatr Infect Dis J . 2019 Feb;38(2):e29-e31http://hdl.handle.net/10400.17/3677Very rarely, patients with X-linked lymphoproliferative syndrome type 1 present central nervous system vasculitis. We report a patient carrying a SH2D1A mutation that, after treatment for lymphoma developed fatal central nervous system vasculitis. He lacked signs of ongoing Epstein-Barr virus infection. We propose that impaired T cell homeostasis caused by SAP deficiency facilitates aberrant CD8 T cell activation against vascular antigens promoting clinical manifestations.engAdolescentAftercareBurkitt LymphomaCentral Nervous System DiseasesFatal OutcomeHumansMaleSignaling Lymphocytic Activation Molecule Associated ProteinVasculitisMutationHDE NEU PEDHDE PEDjournal article10.1097/INF.0000000000002154