DLBCL is more frequent in older individuals (median age is 64 years) and shows a slightly male predominance. Patients with a history of solid organ transplant, immunodeficiency, B-cell-activating autoimmune disorders (ie, systemic lupus erythematosus, Sjögren syndrome, celiac disease), or occupational exposure to agricultural pesticides or ionizing radiation may be at increased risk.
There are several subtypes of DLBCL (as classified by the World Health Organization [WHO]), each of which is characterized by distinct morphology, location, and/or molecular signature. They include:
- Primary mediastinal large B-cell lymphoma
- T cell / histiocyte-rich large B-cell lymphoma
- Intravascular large B-cell lymphoma
- Lymphomatous granulomatosis
- Primary DLBCL of the central nervous system
- Primary cutaneous DLBCL, leg type
- DLBCL associated with chronic inflammation
Patients with DLBCL may present with enlarging lymph nodes (usually in the neck or abdomen, but may be anywhere) or with extranodal disease in the form of a mass. The most common extranodal site for DLBCL to present is the gastrointestinal (GI) tract. Patients may present with B symptoms (ie, fever, weight loss, night sweats), but asymptomatic presentations can occur. Vague, nonspecific symptoms may also occur, as is the case with the rare intravascular B-cell lymphoma. Another uncommon subtype is primary cutaneous DLBCL, leg type. It usually presents as red or violaceous plaques on the lower leg but can occur at any body location. The lesions may quickly develop into tumors that ulcerate.
