Introduction: Chronic active Epstein-Barr virus infection (CAEBV) has been implicated in several diseases including hemophagocytic lymphohistiocytosis (EBV-HLH) and lymphomas including Angioimmunoblastic T cell lymphoma. The exact mechanism by which EBV infection causes these complications is currently not well understood. EBV-HLH is a syndrome in which T-cells, NK cells and macrophages are aberrantly activated. Cytokine storms play a major role in cellular damage and organ dysfunction. In the event that the body is unable to clear the EBV viremia, dysregulated T, NK cells and macrophages continue to release cytokines leading to the accumulation of lymphohistiocytic infiltrates into organs and organ damage. Cytokine storms can lead to death from multi-organ failure. During a cytokine storm, levels of several cytokines are elevated including TNF-a, IFN-g, sCD25, IL-12, IL-1, IL-10 and IL-18.1–3 Current lines of therapy of EBV-HLH include steroids, etoposide, cyclosporine, ATG and hematopoietic stem cell transplantation. We report successfully controlling frequent cytokine storms in a patient with EBV induced angioimmunoblastic lymphoma and HLH with weekly infliximab after failure to do so with chemotherapy (CHOP-E; cyclophosphamide, daunorubicin, vincristine, prednisone, etoposide), rituximab and bortezomib.
Gaballa, Sameh; Besa, Emmanuel; and Alpdogan, Onder, "The Use of Infliximab to Control Recurrent Cytokine Storms in EBV-Associated Angioimmunoblastic Lymphoma and Hemophagocytic Syndrome" (2012). Department of Medical Oncology Faculty Papers. Paper 15.