Abstract
Although sharing some common features in terms of etiology and even clinical presentation, marginal zone lymphoma is a heterogeneous group of diseases, with subtypes that can present in unexpected ways, especially with regard to autoimmune disease. There’s a proven role of antimicrobial therapy for both Helicobacter pylori and hepatitis C virus, and 10-year overall survival is high. Because this disease requires continuous treatment, therapy should be properly sequenced, with consideration given to patient comorbidities and age. Finally, B-cell receptor signaling inhibitors and immunomodulatory agents can provide responses and disease control.