Phase II Study of Interferon-Alpha and DA-EPOCH+/-R in Lymphomatoid Granulomatosis

Background: Lymphomatoid granulomatosis (LYG) is a rare EBV-driven B-cell lymphoproliferative disorder characterized by a reactive T-cell infiltrate that is angioinvasive and angiodestructive. Dysregulated immune surveillance of EBV is thought to contribute to the pathogenesis of LYG and grading of disease is based on the density and number of EBV+ large atypical B-cells. Grade I-II (low-grade) disease is typically polyclonal/oligoclonal and immune-dependent whereas grade III (high-grade) disease is typically monoclonal and immune-independent. Herein, we report additional and extended results from an on-going prospective study at the National Cancer Institute on the treatment of patients with low and high-grade LYG using Interferon-α (INF-α) and/or DA-EPOCH+/-R, respectively.