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. 2022 Jun 3;219(7):e20211682. doi: 10.1084/jem.20211682

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© 2022 Fournier et al.

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Figure 1. — Broad susceptibility to EBV in a patient affected by chronic and disseminated EBV⁺ smooth muscle tumors. (A) Smooth muscle tumor histology at 15 y.o. obtained by splenectomy. Left lower panel, hematoxylin–eosin (HE) staining showing fascicles of spindle-shaped smooth muscle cells with rare interspersed lymphocytes (magnification, ×100). Left upper panel, cytoplasmic staining with anti-caldesmon antibody on tumoral cells (magnification, ×200; scale, 90 μM). Right upper panel, co-staining with an EBER probe and anti-CD3 antibody reveals that tumoral cells are infected by EBV (plain black arrow) while endothelial cells and some infiltrating T cells are negative (white arrows, upper right part and lower left part, respectively; magnification, ×400; scale, 40 μM). Right lower panels, co-staining with an EBER probe and anti-CD79a or anti-CD3 antibodies in the splenic red pulp reveals both infected (black arrows) and non-infected (gray arrows) T and B cells. The white arrow indicates a CD79a-negative infected cell (magnification, ×400; scale, 30 μM). (B) Magnetic resonance images of the EBV⁺ smooth muscle tumors of the left iliac bone at 9 y.o. (left panel) and liver at 17 y.o. (right panel). (C) EBV blood loads in the patient at different ages (years, y; months, m) starting from the first EBV PCR at the age of 9 y.o. (D) FACS dot-plots of EBER expression in PBMCs of the patient at the age of 18 y.o. by primeflow assay coupled with anti-CD19, anti-CD3, anti-CD4, and anti-CD8. Similar findings obtained at 17 y.o. All dot-plots are gated on efluor⁻CD14⁻ cells. (E) FACS dot-plots of co-staining of PBMCs of the patient at 18 y.o. and a control with anti-CD8 and HLA-A02–restricted empty pentamers (left panels) or EBV pentamers (right panels) expression in PBMCs of the patient (Pat.) and control (Ctr.). Cells gated on CD3⁺CD8⁺. No staining was observed in the CD4⁺ cells. Similar findings were obtained with another sample.