Introduction
IVLBCL is a rare form of extranodal diffuse large B-cell lymphoma with an incidence rate of 0.095 per 1,000,0001. Increased recognition of this entity has led to an increase in the number of reported cases over time. Three different subtypes of IVLBCL are identified: classical variant (more common in Western countries), hemophagocytic syndrome-associated (more common in Asian countries), and a cutaneous variant2. Herein, the natural history of IVLBCL is described for four patients, each with a unique presentation of the disease. A brief review of the available literature on this topic is additionally provided.