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. 2022 Aug 18;9(8):ofac351.
doi: 10.1093/ofid/ofac351. eCollection 2022 Aug.

Multisystem T-cell Chronic Active Epstein-Barr Virus Infection: From the Eye to the Kidney

Guillaume Vial  1 , Laure Barthod  1 , Sophie Schneider  1 , Patrick Mercié  1 , Pierre Duffau  1 , Agathe Vermorel  2 , Emmanuel Ribeiro  1
Affiliations

Multisystem T-cell Chronic Active Epstein-Barr Virus Infection: From the Eye to the Kidney

Guillaume Vial et al. Open Forum Infect Dis. .

Abstract

Chronic active Epstein-Barr virus (CAEBV) infection is usually a fatal disease associated with clonal proliferation of EBV-infected T or NK cells. We present the case of a 33-year-old Peruvian patient who developed a multisystem CAEBV, notably responsible for exceptional ophthalmological and renal damage. We describe the clinicopathological features of EBV-induced lymphoproliferative disorder.

Keywords: Epstein-Barr virus; chronic active Epstein-Barr virus infection; lymphoproliferation; orbital myositis; tubulointerstitial nephritis.

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Conflict of interest statement

Potential conflicts of interest. All authors: no reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed.

Figures

Figure 1.
Figure 1.
Pathological and radiological illustrations of EBV-induced lymphoproliferation. Multiorgan dysfunction due to CD3+ CD8+ CD30+ EBER+ cytotoxic T-cell infiltrate. For each biopsy, the CD8+ and EBER+ character is confirmed (A–D). In detail, oral aphthous biopsy showed atypical lymphoproliferation with epitheliotropism corresponding to hydroa vacciniforme-like (A). Liver biopsy revealed sinusoidal and portal infiltrate (B). Renal biopsy showed a massive tubulointerstitial infiltrate without glomerulopathy or vasculopathy (C). Skin biopsy showed a diffuse nodular infiltrate of the dermis and hypodermis (D). Axial (low) and coronal (top) contrast-enhanced T1 magnetic resonance imaging of the orbits showed multilocal edema and enlargement of the various ocular muscles (E). Chest CT showed pleural and pericardial effusion (F). Abbreviations: CT, computed tomography; EBV, Epstein-Barr virus.
See this image and copyright information in PMC

References

    1. Kimura H, Ito Y, Kawabe S, et al. . EBV-associated T/NK-cell lymphoproliferative diseases in nonimmunocompromised hosts: prospective analysis of 108 cases. Blood 2012; 119:673–86. - PubMed
    1. Cohen JI, Jaffe ES, Dale JK, et al. . Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States. Blood 2011; 117:5835–49. - PMC - PubMed
    1. Fujiwara S, Nakamura H. Chronic active Epstein-Barr virus infection: is it immunodeficiency, malignancy, or both? Cancers (Basel) 2020; 12:3202. - PMC - PubMed
    1. Okano M, Kawa K, Kimura H, et al. . Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection. Am J Hematol 2005; 80:64–9. - PubMed
    1. Kimura H, Cohen JI. Chronic active Epstein-Barr virus disease. Front Immunol 2017; 8:1867. - PMC - PubMed