Fatal Central Nervous System Lymphocytic Vasculitis after Treatment for Burkitt Lymphoma in a Patient with a SH2D1A Mutation

Neves, JF; Raga, LT; Chiang, SCC; Tesi, B; Vieira, JP; Cordeiro, AI; Borrego, L; Bryceson, YT

http://hdl.handle.net/10400.17/3677

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Abstract(s)

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.

Keywords

Adolescent Aftercare Burkitt Lymphoma Central Nervous System Diseases Fatal Outcome Humans Male Signaling Lymphocytic Activation Molecule Associated Protein Vasculitis Mutation HDE NEU PED HDE PED