Patients with Castleman disease report mild COVID-19 symptoms and mount a humoral response to SARS-CoV-2 vaccination

Abstract

The coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection has resulted in increased morbidity and mortality in patients with impaired immunity, hematologic malignancies, and on immunosuppressive regimens. COVID-19 can cause a cytokine storm with some patients benefiting from blockade of the proinflammatory cytokine, interleukin 6 (IL-6). Because Castleman disease (CD) is an atypical lymphoproliferative disorder that can involve a cytokine storm and often requires immunosuppressive therapies, including IL-6 inhibition, we sought to evaluate outcomes after COVID-19 and SARS-CoV-2 vaccination in patients with CD. We administered a survey in April 2021 to characterize experiences with COVID-19 and SARS-CoV-2 vaccination among 300 patients enrolled in ACCELERATE, a CD natural history registry. Among 128 respondents, the prevalence of SARS-CoV-2 infection (16/95, 17%), severe disease (1/16, 6%), vaccination rates (112/128, 88%), and vaccine adverse effects after dose 1 (62/112, 55%) were comparable with that of the general US population. Although there were 2 cases of CD flares occurring shortly after SARS-CoV-2 infection (n = 1) and vaccination (n = 1), >100 patients that were infected and/or vaccinated did not experience CD flares. Among patients with CD, the median antispike titer 6 months after the second vaccine dose was comparable to that of individuals with other immune-related diseases and healthy populations. Despite being on immunosuppressive therapies, patients with CD do not appear to be at increased risk of poor COVID-19 outcomes and can mount a humoral response to SARS-CoV-2 vaccination. This study was registered at www.ClinicalTrials.gov as #NCT02817997.