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

Pediatr Infect Dis J. 2019 Feb;38(2):e29-e31. doi: 10.1097/INF.0000000000002154.

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 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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aftercare
  • Burkitt Lymphoma / complications*
  • Burkitt Lymphoma / pathology
  • Burkitt Lymphoma / therapy
  • Central Nervous System Diseases / diagnosis*
  • Central Nervous System Diseases / pathology*
  • Fatal Outcome
  • Humans
  • Male
  • Mutation*
  • Signaling Lymphocytic Activation Molecule Associated Protein / genetics*
  • Vasculitis / diagnosis*
  • Vasculitis / pathology*

Substances

  • SH2D1A protein, human
  • Signaling Lymphocytic Activation Molecule Associated Protein