PMC8113761_01
(A) Microcephaly (B) Gastric biopsy: EBV-encoded small ribonucleic acid (EBER1) in situ hybridization shows positive nuclei EBER+ in the T cells lymphocytes. Double staining EBER-ISH (blue) and CD3-IHC (brown) (C) Thorax CT with mediastinal infiltration. (D) Clinical evolution, EBV load and therapeutics.
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A gastric ulcer biopsy confirmed a polymorphic T infiltrate (EBER+) ( Figure 1B ) and rearrangements of T cell receptors and flow cytometry excluded monoclonality. He was treated with rituximab for B lymphocyte depletion with resolution of fever and of the gastrointestinal bleeding, which were accompanied by marked decrease in the EBV viral load (viral load 50,000> 1015 copies/mL ( Figure 1D ). T-cell LPD, and despite the absence of documented T-cell infection in the peripheral blood, cyclophosphamide was added to rituximab when the patient eventually presented a disease relapse, with fever, increasing viral load and generalized lymphadenopathies. This led to a dramatic clinical improvement and a decrease in viral load to 20 copies/ml ( Figure 1D ). Unfortunately, in the following months, the patient had several infectious episodes (namely central line related sepsis), leading to intermittent discontinuation of the immunosuppressive treatment and subsequent relapse of the disease with massive lymphoproliferation ( Figure 1C ) and increasing viral loads, eventually leading to his death.