Diffuse large B-cell lymphoma

Diffuse large B cell lymphoma
Other namesDLBCL or DLBL
Micrograph (Field stain) of a diffuse large B cell lymphoma.
SpecialtyHematology, oncology, dermatology

Diffuse large B-cell lymphoma (DLBCL) is a cancer of B cells, a type of lymphocyte that is responsible for producing antibodies. It is the most common form of non-Hodgkin lymphoma among adults,[1] with an annual incidence of 7–8 cases per 100,000 people per year in the US and UK.[2][3] This cancer occurs primarily in older individuals, with a median age of diagnosis at ~70 years,[3] although it can occur in young adults and, in rare cases, children.[4] DLBCL can arise in virtually any part of the body and, depending on various factors, is often a very aggressive malignancy.[5] The first sign of this illness is typically the observation of a rapidly growing mass or tissue infiltration that is sometimes associated with systemic B symptoms, e.g. fever, weight loss, and night sweats.[6]

The causes of diffuse large B-cell lymphoma are not well understood. Usually DLBCL arises from normal B cells, but it can also represent a malignant transformation of other types of lymphoma (particularly marginal zone lymphomas[7]) or, in rare cases termed Richter's transformation, chronic lymphocytic leukemia.[8] An underlying immunodeficiency is a significant risk factor for development of the disease.[9] Infections with the Epstein–Barr virus (EBV),[10][11] Kaposi's sarcoma-associated herpesvirus,[12][13] human immunodeficiency virus (i.e. HIV),[12] and the Helicobacter pylori bacterium[7] are also associated with the development of certain subtypes of diffuse large B-cell lymphoma. However, most cases of this disease are associated with the unexplained step-wise acquisition of increasing numbers of gene mutations and changes in gene expression that occur in, and progressively promote the malignant behavior of, certain B-cell types.[14]

Diagnosis of DLBCL is made by removing a portion of the tumor through a biopsy, and then examining this tissue using a microscope. Usually a hematopathologist makes this diagnosis.[15] Numerous subtypes of DLBCL have been identified which differ in their clinical presentations, biopsy findings, aggressive characteristics, prognoses, and recommended treatments.[16] However, the usual treatment for most subtypes of DLBCL is chemotherapy combined with a monoclonal antibody drug that targets the disease's cancerous B-cells, usually rituximab.[17] Through these treatments, more than half of all patients with DLBCL can be cured;[18] the overall cure rate for older adults is less than this but their five-year survival rate has been around 58%.[19]

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  7. ^ a b Casulo C, Friedberg J (2017). "Transformation of marginal zone lymphoma (and association with other lymphomas)". Best Practice & Research. Clinical Haematology. 30 (1–2): 131–138. doi:10.1016/j.beha.2016.08.029. PMID 28288708.
  8. ^ Abuelgasim KA, Rehan H, Alsubaie M, Al Atwi N, Al Balwi M, Alshieban S, Almughairi A (March 2018). "Coexistence of chronic myeloid leukemia and diffuse large B-cell lymphoma with antecedent chronic lymphocytic leukemia: a case report and review of the literature". Journal of Medical Case Reports. 12 (1): 64. doi:10.1186/s13256-018-1612-4. PMC 5845776. PMID 29524963.
  9. ^ Swerdlow et al. 2008, p. 233.
  10. ^ Castillo JJ, Beltran BE, Miranda RN, Young KH, Chavez JC, Sotomayor EM (July 2018). "EBV-positive diffuse large B-cell lymphoma, not otherwise specified: 2018 update on diagnosis, risk-stratification and management". American Journal of Hematology. 93 (7): 953–962. doi:10.1002/ajh.25112. hdl:20.500.12727/6261. PMID 29984868.
  11. ^ Nicolae A, Pittaluga S, Abdullah S, Steinberg SM, Pham TA, Davies-Hill T, Xi L, Raffeld M, Jaffe ES (August 2015). "EBV-positive large B-cell lymphomas in young patients: a nodal lymphoma with evidence for a tolerogenic immune environment". Blood. 126 (7): 863–72. doi:10.1182/blood-2015-02-630632. PMC 4536540. PMID 25999451.
  12. ^ a b Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ (November 2019). "Diffuse large B-cell lymphoma variants: an update". Pathology. 52 (1): 53–67. doi:10.1016/j.pathol.2019.08.013. PMID 31735345. S2CID 208142227.
  13. ^ Shimada K, Hayakawa F, Kiyoi H (November 2018). "Biology and management of primary effusion lymphoma". Blood. 132 (18): 1879–1888. doi:10.1182/blood-2018-03-791426. PMID 30154110.
  14. ^ Liu Y, Barta SK (May 2019). "Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment". American Journal of Hematology. 94 (5): 604–616. doi:10.1002/ajh.25460. PMID 30859597.
  15. ^ Goldman & Schafer 2012, p. 1222.
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