Two cases of aquaporin-4 positive neuromyelitis optica associated with T-cell lymphoma

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Background: Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory condition associated with antibodies against aquaporin-4 (AQP4). To date, 4 cases of B-cell lymphomas have been reported in patients with NMOSD. Here we describe two individuals with AQP4+ NMOSD and T-cell lymphomas (TCL). Conclusion: Possible mechanisms that may explain co-existence of NMOSD with TCL include a paraneoplastic syndrome leading to AQP4 antibody production, or a generalized dysfunction of the adaptive immune system. Both patients were diagnosed with NMOSD and initiated on anti-CD20 agents prior to lymphoma diagnosis, though the short duration of exposure argues against a direct role. Further research is needed to examine the rate of lymphoproliferative disorders in NMOSD.

Original languageEnglish (US)
Article number577092
JournalJournal of Neuroimmunology
Publication statusPublished - Jan 15 2020


ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Neurology
  • Clinical Neurology

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