Kyle Blackburn1, Cynthia Wang2, Benjamin Greenberg2. 1. University of Texas Southwestern Medical Center, Department of Neurology and Neurotherapeutics, 5323 Harry Hines Blvd, Dallas, TX 75390, United States. Electronic address: kyle.blackburn@utsouthwestern.edu. 2. University of Texas Southwestern Medical Center, Department of Neurology and Neurotherapeutics, 5323 Harry Hines Blvd, Dallas, TX 75390, United States; University of Texas Southwestern Medical Center, Department of Pediatrics, 5323 Harry Hines Blvd, Dallas, TX 75390, United States.
Abstract
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.
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.
Authors: Pilar Carrillo; Teresa Gorría; Daniel Santana; Maria Sepulveda; Iban Aldecoa; Blanca Gonzalez-Farré; Esther Sanfeliu; Eduard Mension; Isaac Cebrecos; Olga Martínez-Saez; Imma Alonso; Albert Saiz Journal: Biomed Hub Date: 2022-01-31