Article
Fatal Central Nervous System Lymphocytic Vasculitis after Treatment for Burkitt Lymphoma in a Patient with a SH2D1A Mutation.
The Pediatric infectious disease journal - 1 Feb 2019
Neves João Farela, Raga Lamberto Torralba, Chiang Samuel C C, Tesi Bianca, Vieira José Pedro, Cordeiro Ana Isabel, Borrego Luis, Bryceson Yenan T
Abstract excerpt
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...
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