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

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 infect