Fatal central nervous system lymphocytic vasculitis after treatment for Burkitt lymphoma in a patient with a SH2D1A mutation

João Farela Neves, Lamberto Torralba Raga, Samuel C.C. Chiang, Bianca Tesi, José Pedro Vieira, Ana Isabel Cordeiro, Luis Borrego, Yenan T. Bryceson
2018 The Pediatric Infectious Disease Journal  
Very 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.
doi:10.1097/inf.0000000000002154 pmid:30138256 fatcat:2p7gquxgoffqjd3fizdr3n5tx4