"Abstract
Hemophagocytic lymphohistiocytosis (HLH) has been recognized as a rare adverse event following the coronavirus disease 2019 (COVID-19) vaccination. We report a case of neuropsychiatric symptoms and refractory HLH in a woman with systemic lupus erythematosus (SLE) after receiving her COVID-19 vaccine treated with belimumab, later found to have intravascular large B-cell lymphoma (IVLBCL) at autopsy... Despite treatment, the patient died on day 75; autopsy report findings suggested IVLBCL as the underlying cause of HLH. Differentiating comorbid conditions remains difficult; however, in the case of an atypical clinical presentation, other causes should be considered. Therefore, we speculate that the COVID-19 vaccination and her autoimmune condition may have expedited IVLBCL development...
1. Introduction
... HLH is a life-threatening hyperinflammatory syndrome exhibiting atypical histiocyte proliferation triggered by various conditions, such as infections, malignancy and autoimmune disorders...
3. Discussion
... [S]everal studies have reported SLE symptoms exacerbation post-vaccination. Felten et al. evaluated the prevalence of COVID-19 vaccination side effects in SLE patients through a web-based survey, showing that 21 of 696 patients (3%) reported an SLE flare after a median of 3 days. In these patients, the predominant symptoms were as follows: musculoskeletal symptoms, fatigue, cutaneous flares, fever and cytopenia... Izmirly reported that 9 of 79 patients experienced a disease flare after vaccination...
Several reports have been published on the association between the COVID-19 vaccine and malignant lymphoma. Panou et al. reported two cutaneous T-cell lymphoma relapse cases where patients had been in remission for several years and relapsed after viral vector COVID-19 vaccination. Brumfiel et al. reported a cutaneous CD30-positive lymphoma relapse case after mRNA COVID-19 vaccination. Gambichler et al. reported a patient with primary cutaneous anaplastic large-cell lymphoma with complete remission that exacerbated one week after the first mRNA COVID-19 vaccination. These reports imply that COVID-19 vaccination induces existing T-cell lymphoma recurrence. Goldman et al. described a patient who developed angioimmunoblastic T-cell lymphoma after two BNT162b2 mRNA vaccine rounds, exacerbated by a third booster dose. These reports indicate a relationship between COVID-19 vaccination and T-cell lymphomagenesis. Our patient, in contrast, developed B-cell lymphoma...
A previous meta-analysis showed an increased malignancy risk in SLE patients."
© 2024 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group on behalf of the Japanese Society of Clinical Immunology.
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