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Case Reports
. 2022 Oct 15;10(10):1725.
doi: 10.3390/vaccines10101725.

Idiopathic Multicentric Castleman Disease Occurring Shortly after mRNA SARS-CoV-2 Vaccine

Christian Hoffmann  1   2 , Thomas Wechselberger  3 , Heinz Drexel  3   4 , Susanne Dertinger  5 , Stefan Dirnhofer  6 , Sheila K Pierson  7 , David C Fajgenbaum  7 , Andreas Kessler  3
Affiliations
Case Reports

Idiopathic Multicentric Castleman Disease Occurring Shortly after mRNA SARS-CoV-2 Vaccine

Christian Hoffmann et al. Vaccines (Basel). .

Abstract

Idiopathic Multicentric Castleman Disease (iMCD) is a potentially life-threatening systemic disease whose complex symptomatology is due to cytokine dysregulation. We, herein, present a case of severe iMCD occurring in a previously healthy young man shortly after mRNA SARS-CoV-2 vaccination, responding to interleukin-6 blockade with siltuximab. Six months after the completion of siltuximab, the patient remained without any signs of iMCD or inflammation, indicating a temporal trigger of the disease. This case not only adds to the potential pathogenetic spectrum of MCD, but also extends the clinical picture of potential but rare adverse events following COVID-19 immunization.

Keywords: Idiopathic Multicentric Castleman Disease; TAFRO syndrome; mRNA SARS-CoV-2 vaccine; siltuximab.

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Conflict of interest statement

All the authors certify that they have no affiliations with/or involvement in any organization or entity with any financial interest in the subject matter or materials discussed in this manuscript.

Figures

Figure 1
Figure 1
(a) Reticulin stain of bone marrow showing focally increased megakaryopoiesis (e.g., blue arrows) with little fibrosis (e.g., black arrow) (×40). (b) Regressed germinal centers (rGCs), interfollicular high endothelial vessels (HEV) and plasmocytosis (hematoxylin and eosin staining × 200). (c) “Onion skinning” created by expanded mantle zones with concentrically arranged lymphocytes around the regressed GC (hematoxylin and eosin staining × 100). (d) Immunohistochemistry (ICH, × 100) staining for kappa (right) and lambda light chains (left). Ig Kappa:Ig Lambda = 1:5.
Figure 1
Figure 1
(a) Reticulin stain of bone marrow showing focally increased megakaryopoiesis (e.g., blue arrows) with little fibrosis (e.g., black arrow) (×40). (b) Regressed germinal centers (rGCs), interfollicular high endothelial vessels (HEV) and plasmocytosis (hematoxylin and eosin staining × 200). (c) “Onion skinning” created by expanded mantle zones with concentrically arranged lymphocytes around the regressed GC (hematoxylin and eosin staining × 100). (d) Immunohistochemistry (ICH, × 100) staining for kappa (right) and lambda light chains (left). Ig Kappa:Ig Lambda = 1:5.
Figure 2
Figure 2
C-reactive protein (CRP, mg/L over time and siltuximab infusions (arrows).
See this image and copyright information in PMC

References

    1. Angeli F., Spanevello A., Reboldi G., Visca D., Verdecchia P. SARS-CoV-2 vaccines: Lights and shadows. Eur. J. Intern. Med. 2021;88:1–8. doi: 10.1016/j.ejim.2021.04.019. - DOI - PMC - PubMed
    1. Fajgenbaum D.C. Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018;132:2323–2330. doi: 10.1182/blood-2018-05-848671. - DOI - PMC - PubMed
    1. Nishimora Y., Fajgenbaum D.C., Pierson S.K., Iwaki N., Nishikori A., Kawano M., Nakamura N., Izutsu K., Takeuchi K., Nishimura M.F., et al. Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease. Am. J. Hematol. 2021;96:1241–1252. doi: 10.1002/ajh.26292. - DOI - PMC - PubMed
    1. van Rhee F., Voorhees P., Dispenzieri A., Fosså A., Srkalovic G., Ide M., Munshi N., Schey S., Streetly M., Pierson S.K., et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132:2115–2124. doi: 10.1182/blood-2018-07-862334. - DOI - PMC - PubMed
    1. van Rhee F., Wong R.S., Munshi N., Rossi J.-F., Ke X., Fosså A., Simpson D., Capra M., Liu T., Hsieh E.K., et al. Siltuximab for multicentric Castleman’s disease: A randomised, double-blind, placebo-controlled trial. Lancet Oncol. 2014;15:966–974. doi: 10.1016/S1470-2045(14)70319-5. - DOI - PubMed

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