Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin lymphoma.
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Epidemiology
Associations
Diffuse large B-cell lymphoma is sometimes associated with immunodeficiency, including acquired immunodeficiency syndrome (AIDS).
Pathology
Classification
In the 2016 WHO classification of lymphoid neoplasms, diffuse large B-cell lymphoma, not otherwise specified (NOS), is a type of mature B-cell neoplasm and includes two molecular subgroups indicating the cell of origin:
germinal centre B-cell type
activated B-cell type
In addition, diffuse large B-cell lymphomas with certain molecular/cytogenetic features, previously called "double-hit" or "triple-hit" lymphomas, as well as those resembling Burkitt lymphoma are now classified together as either of the following entities 1:
There are otherwise multiple specified types of diffuse large B-cell lymphomas:
primary diffuse large B-cell lymphoma of the central nervous system
Epstein-Barr virus-positive diffuse large B-cell lymphoma, not otherwise specified
diffuse large B-cell lymphoma associated with chronic inflammation
human herpesvirus 8-positive diffuse large B-cell lymphoma, not otherwise specified (provisional entity)
Staging
See the article on lymphoma staging.
Treatment and prognosis
Considered an "aggressive" lymphoma, DLBCL tends to show good response to chemotherapy. Typically, this involves the R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen and approximately two-thirds of patients show response to this. For those that have refractory or relapsing disease, treatment involves salvage chemotherapy with autologous stem cell transplantation. Chimeric antigen receptor (CAR)-T cell therapy can be used in those who then fail this second-line therapy 2.
Complications
When affecting the gastrointestinal tract, DLBCL carries a higher is a risk of bowel perforation than other non-Hodgkin's lymphomas 3,4.
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