Angioimmunoblastic T-Cell Lymphoma with Concurrent EBV Viremia: A Challenging Case

Abstract

Abstract Introduction/Objective Angioimmunoblastic T-cell lymphoma (AITL) represents only 1-2% of all non-Hodgkin lymphomas but is one of the most common non-cutaneous T-cell lymphomas. Although typically associated with latent Epstein Barr Virus (EBV) infection, recent studies have demonstrated the presence and prognostic impact of EBV viremia at the time of diagnosis. Here, we present a case of AITL in which the diagnosis was complicated by EBV viremia. Methods/Case Report A 41-year-old male presented to our hospital with generalized abdominal pain, diffuse lymphadenopathy, hepatomegaly, ascites, and diffuse body wall edema. A recent retroperitoneal lymph node biopsy reportedly demonstrated features suggesting an infectious process, and EBV PCR revealed a viral load of 141,050 copies/mL. A left axillary lymph node was excised, which demonstrated vascular proliferation and diffuse architectural effacement by a polymorphous infiltrate including variably sized lymphocytes with moderate to abundant clear cytoplasm, and occasional admixed neutrophils, plasma cells, and eosinophils. Immunohistochemical staining demonstrated an atypical CD4-positive, CD7-negative T-cell population in a vaguely nodular distribution, expressing Bcl-6, PD-1, ICOS, and CXCL13 without definite CD10 expression. CD21 immunostaining demonstrated markedly expanded follicular dendritic cell meshworks in a distribution similar to the atypical T-cell population. No B-cell or cytotoxic T-cell proliferation was seen, and in situ hybridization for EBV-encoded RNA (EBER ISH) demonstrated only scattered EBV- positive cells. Taken together, these findings supported a diagnosis of AITL over an atypical reaction to EBV infection. Next-generation sequencing demonstrated mutations in TET2, IDH2, and RHOA, further confirming the diagnosis. Results (if a Case Study enter NA) NA Conclusion AITL manifests with a diverse range of clinical symptoms and pathological features and may mimic infection. Additionally, acute EBV infection is well-known to mimic various lymphomas and has rarely been reported to mimic AITL. We present this case to raise awareness of the concurrent presentation of AITL with EBV viremia and to highlight features that may be helpful in distinguishing between the two.