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

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

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 CNS vasculitis. He lacked signs of ongoing EBV infection. We propose that impaired T cell homeostasis caused by SAP-deficiency facilitates aberrant CD8 T cell activation against vascular antigens promoting clinical manifestations.

Original languageEnglish
Pages (from-to)e29-e31
JournalPediatric Infectious Disease Journal
Volume38
Issue number2
Early online date22 Aug 2018
DOIs
Publication statusPublished - Feb 2019

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