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Abstract Details

Late Relapse After Prolonged Remission Post-autologous Hematopoietic Stem Cell Transplantation in Two Patients with AQP4-IgG+ Neuromyelitis Optica Spectrum Disorder
Autoimmune Neurology
P11 - Poster Session 11 (5:30 PM-6:30 PM)
14-001

A previous study on non-myeloablative autologous hematopoietic stem cell transplantation (AHSCT) in patients with neuromyelitis optica spectrum disorder (NMOSD) reported seroreversion from AQP4-IgG + to - status and up to five years of remission [Neurology. 2019;93(18):e1732-e1741]. Longitudinal follow-up data beyond five years are lacking, and patients may become lost to follow-up.

 

To report the long-term outcomes of two NMOSD patients post-transplant from the previous report.

We conducted clinical record reviews and measured AQP4-IgG status and cell-killing activity using live AQP4-transfected cells by flow cytometry.

The first patient, a 73-year-old woman diagnosed with NMOSD in January 2010 after presenting with longitudinal extensive transverse myelitis (LETM) and testing positive for AQP4-IgG (titer 100), experienced a LETM relapse in April 2010. After AHSCT in October 2010, her AQP4-IgG seroreverted to negative status, and she remained relapse-free for 12 years. However, she later relapsed with area postrema syndrome, and her AQP4-IgG seroconverted to positive (titer 1000). She made a good recovery following steroid treatment and commenced Eculizumab. The second patient, a 60-year-old woman, experienced multiple episodes of optic neuritis dating back to November 2002, with an AQP4-IgG titer of 1:100 in 2008. Despite receiving rituximab, she had relapses of LETM from 2010-2012. After AHSCT in February 2013, her AQP4-IgG seroreverted to negative status, and she remained relapse-free for 10 years. However, she later relapsed with LETM, and her AQP4-IgG seroconverted to positive (titer >100,000). Unfortunately, she developed pulmonary aspergillosis and passed away in September 2023.

Patients undergoing AHSCT for AQP4+ NMOSD who experience seroreversion (+ to - AQP4-IgG status) and remain attack-free for 10 years or more may eventually seroconvert (- to +) and relapse. This study highlights the necessity for continued clinical and serological surveillance over the long term in NMOSD patients post-AHSCT.

Authors/Disclosures
Nisa Vorasoot, MD (Department of Neurology, Mayo Clinic)
PRESENTER
Dr. Vorasoot has nothing to disclose.
Kyle M. Blackburn, MD (University of Texas Southwestern Medical Center) Dr. Blackburn has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics.
Jiraporn Jitprapaikulsan, MD (Faculty of Medicine Siriraj Hospital) Dr. Jitprapaikulsan has nothing to disclose.
Melissa Bush (Mayo Clinic) Mrs. Bush has nothing to disclose.
James Fryer James Fryer has received intellectual property interests from a discovery or technology relating to health care.
Sean J. Pittock, MD (Mayo Clinic Dept of Neurology) Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech, Inc.. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics, Inc.. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Prime Therapeutics. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MedImmune/Viela Bio. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche/Genentech. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB, Inc. Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffman/LaRoche AG. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for F. Hofman/LaRoche. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. The institution of Dr. Pittock has received research support from Grifols. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Viela Bio/MedImmune/Horizon. The institution of Dr. Pittock has received research support from Alexion Pharmaceuticals. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. The institution of Dr. Pittock has received research support from NovelMed. The institution of Dr. Pittock has received research support from AstaZeneca. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.