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

2019Journal article Open access
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dc.contributor.authorNeves, JF
dc.contributor.authorRaga, LT
dc.contributor.authorChiang, SCC
dc.contributor.authorTesi, B
dc.contributor.authorVieira, JP
dc.contributor.authorCordeiro, AI
dc.contributor.authorBorrego, L
dc.contributor.authorBryceson, YT
dc.date.accessioned2021-04-29T18:55:55Z
dc.date.available2021-04-29T18:55:55Z
dc.date.issued2019
dc.description.abstractVery 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.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationPediatr Infect Dis J . 2019 Feb;38(2):e29-e31pt_PT
dc.identifier.doi10.1097/INF.0000000000002154pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.17/3677
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherWolters Kluwer Healthpt_PT
dc.subjectAdolescentpt_PT
dc.subjectAftercarept_PT
dc.subjectBurkitt Lymphomapt_PT
dc.subjectCentral Nervous System Diseasespt_PT
dc.subjectFatal Outcomept_PT
dc.subjectHumanspt_PT
dc.subjectMalept_PT
dc.subjectSignaling Lymphocytic Activation Molecule Associated Proteinpt_PT
dc.subjectVasculitispt_PT
dc.subjectMutationpt_PT
dc.subjectHDE NEU PEDpt_PT
dc.subjectHDE PEDpt_PT
dc.titleFatal Central Nervous System Lymphocytic Vasculitis after Treatment for Burkitt Lymphoma in a Patient with a SH2D1A Mutationpt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPagee31pt_PT
oaire.citation.issue2pt_PT
oaire.citation.startPagee29pt_PT
oaire.citation.titleThe Pediatric infectious disease journalpt_PT
oaire.citation.volume38pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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