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

doi:10.1097/INF.0000000000002154

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

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5 Citations (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 language	English
Pages (from-to)	e29-e31
Journal	Pediatric Infectious Disease Journal
Volume	38
Issue number	2
Early online date	22 Aug 2018
DOIs	https://doi.org/10.1097/INF.0000000000002154
Publication status	Published - Feb 2019

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

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.",

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AU - Neves, João Farela

AU - Raga, Lamberto Torralba

AU - Chiang, Samuel C C

AU - Tesi, Bianca

AU - Vieira, José Pedro

AU - Cordeiro, Ana Isabel

AU - Borrego, Luis

AU - Bryceson, Yenan T

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N2 - 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.

AB - 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.

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DO - 10.1097/INF.0000000000002154

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SN - 0891-3668

VL - 38

SP - e29-e31

JO - Pediatric Infectious Disease Journal

JF - Pediatric Infectious Disease Journal

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ER -

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

Abstract

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