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.