Abstract: PUB302
Epstein-Barr Virus-Driven Lymphoproliferative Disorder: Renal Sequelae of a Viral Oncogenic Storm
Session Information
Category: Onconephrology
- 1700 Onconephrology
Authors
- Rohrer, Alex, Northeast Ohio Medical University, Rootstown, Ohio, United States
- Gaur, Mragank, University of Cincinnati, Cincinnati, Ohio, United States
- Gudsoorkar, Prakash Shashikant, University of Cincinnati, Cincinnati, Ohio, United States
Introduction
Epstein–Barr virus (EBV) infects >95 % of humans, remaining latent (IgG +) and typically silent. EBV-driven lymphoproliferative disease (LPD) can injure kidneys; we report an elderly man with EBV viremia–linked AKI.
Case Description
77-year-old male (diabetes & hypertension) presented with 2 weeks of weight loss, fever, and morbilliform rash for 2 days. Labs (table 1): leukocytosis with atypical lymphocytes, and AKI needing hemodialysis (3 sessions). Immunological workup showed clonal B cell population on flow cytometry. Protein electrophoresis showed 2 M-spikes (IgM kappa, 0.7 g/dL, and IgG lambda, 1.0 g/dL). Infectious workup: marked EBV viremia with negative monospot test and serologies. Positron Emission Tomography (PET)-CT: multifocal FDG-avid uptake in lymph nodes, marrow, and splenomegaly concerning for LPD. Kidney biopsy (Fig 1&2): acute tubulointerstitial nephritis with EBV in situ hybridization stain (ISH) positive in scattered lymphocytes in the interstitium. Liver biopsy: atypical sinusoidal T cell proliferation with scattered B cells showing EBV-encoded small RNAs (EBER) positivity on ISH. BM biopsy: atypical T-cell proliferation, increased EBER-positive B-cells, and focal hemophagocytic activity (no systemic HLH) . Next-generation sequencing showed T-cell receptor gamma gene rearrangement. A diagnosis of Peripheral T cell lymphoma, NOS, involving liver and BM was made and treated with 4 cycles of CHOP (cyclophosphamide, Hydroxydaunorubicin, Vincristine, Prednisone). At ten months, his kidney & liver function & hematology labs are stable (table 1) with no evidence of lymphoma.
Discussion
Multi-organ EBV-LPD is hard to diagnose and treat, requiring thorough pathology to rule out other lymphoid neoplasms. While systemic EBV-related T-cell lymphoma with HLH usually carries a grim prognosis, our patient responded well.