Diffuse large B cell lymphoma (DLBCL) is the most common histologic subtype of non-Hodgkin lymphoma (NHL), accounting for approximately 30 percent of patients with NHL [1-4]. It is an aggressive NHL in which survival without treatment is measured in months. (See "Classification of the hematopoietic neoplasms".)
The initial treatment of DLBCL is dependent upon the extent of disease. For patients with NHL, disease stage is determined using the Ann Arbor staging system with Cotswold modification, originally developed for Hodgkin lymphoma. This staging system focuses on the number of tumor sites (nodal and extranodal), location, and the presence or absence of systemic ("B") symptoms (table 1). For treatment purposes, patients with DLBCL are generally classified as having either limited stage disease or advanced stage disease based upon whether or not the tumor can be contained within one irradiation field:
●Limited stage disease (usually Ann Arbor stage I or II) — Limited stage DLBCL can be contained within one irradiation field. This population accounts for 30 to 40 percent of patients with DLBCL. Limited stage DLBCL is treated primarily with combined modality therapy consisting of abbreviated systemic chemotherapy (three cycles), the recombinant anti-CD20 antibody rituximab, and involved field radiation therapy. Alternatively, full course (six to eight cycles) systemic chemotherapy plus rituximab without radiation therapy may be used.
●Advanced stage disease (usually Ann Arbor stage III or IV) — Advanced stage DLBCL cannot be contained within one irradiation field. This population accounts for 60 to 70 percent of patients with DLBCL. Advanced stage DLBCL is treated with systemic chemotherapy plus the recombinant anti-CD20 antibody rituximab. (See "Initial treatment of advanced stage diffuse large B cell lymphoma".)
Patients with bulky (>10 cm) stage II disease and patients with stage IIB disease have a less favorable prognosis than those with non-bulky stage II disease without systemic B symptoms. Many clinicians treat such patients in a similar fashion to those with advanced stage disease. (See 'Bulky disease' below.)