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