Diffuse large B cell lymphoma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
Overview
Diffuse large B-cell lymphoma (DLBCL or DLBL) is a cancer of B cells, a type of white blood cell responsible for producing antibodies. Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on molecular, genetic, and immunohistochemical features into more than 20 subgroups. Diffuse large B cell lymphoma may be classified into 2 subtypes based on gene expression profiles. The progression to diffuse large B cell lymphoma involves the microRNAs (miRNAs). On microscopic histopathological analysis, diffuse large B cell lymphoma can be divided into three variants: centroblastic, immunoblastic, and anaplastic. There are no established causes for diffuse large B cell lymphoma. Diffuse large B cell lymphoma is the most common type of non-Hodgkin lymphoma among adults,[1] with an annual incidence of 7–8 cases per 100,000 people per year.[2][3]This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age,[3] though it can also occur in children and young adults in rare cases.[4]The most potent risk factor in the development of diffuse large B cell lymphoma is immunodeficiency. Diffuse large B cell lymphoma must be differentiated from other diseases such as follicular lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma(MALT), small cell lymphocytic lymphoma, and Mantle cell lymphoma (MCL). The prognosis of diffuse large B cell lymphoma depends on the stage of the disease. Diffuse large B cell lymphoma is associated with a 5 year survival rate ranging from 70% to more than 90% among children. According to the Lugano classification, there are four stages of diffuse large B cell lymphoma based on the number of nodes involved and extranodal status. The most common symptoms of diffuse large B cell lymphoma include a rapidly enlarging, non-painful mass that may be located in neck, groin or abdomen. The less common symptoms of diffuse large B cell lymphoma include fever, weight loss and drenching night sweats. Common physical examination findings of diffuse large B cell lymphoma include a non tender mass which can arise in virtually any part of the body.Laboratory tests of diffuse large B cell lymphoma include cytology, genetic testing, histologic confirmation, and immunophenotyping. CT scan is helpful in the diagnosis of diffuse large B cell lymphoma. Lymph node or extranodal tissue biopsy is diagnostic of large B cell lymphoma. The optimal therapy for diffuse large B cell lymphoma depends on the stage at diagnosis. The predominant therapy for diffuse large B cell lymphoma is chemotherapy. Adjunctive radiotherapy may be required. Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.
Classification
Diffuse large B cell lymphoma may be classified based on location into nodal and extranodal disease and based on molecular, genetic, and immunohistochemical features into more than 20 subgroups.
Pathophysiology
Diffuse large B cell lymphoma may be classified into 2 subtypes based on gene expression profiles. The progression to diffuse large B cell lymphoma involves the microRNAs (miRNAs). On microscopic histopathological analysis, diffuse large B cell lymphoma can be divided into three variants: centroblastic, immunoblastic, and anaplastic.
Causes
There are no established causes for diffuse large B cell lymphoma.
Differential diagnosis
Diffuse large B cell lymphoma must be differentiated from other diseases such as follicular lymphoma, Mucosa-Associated Lymphatic Tissue lymphoma(MALT), small cell lymphocytic lymphoma, and mantle cell lymphoma (MCL).
Epidemiology and demographics
Diffuse large B cell lymphoma is the most common type of non-Hodgkin lymphoma among adults,[1] with an annual incidence of 7–8 cases per 100,000 people per year[2][3]. This cancer occurs primarily in older individuals, with a median age of diagnosis at approximately 70 years of age,[3] though it can also occur in children and young adults in rare cases[4].
Risk factors
The most potent risk factor in the development of diffuse large B cell lymphoma is immunodeficiency.
Screening
Screening for diffuse large B cell lymphoma is not recommended.
Prognosis
The prognosis of diffuse large B cell lymphoma depends on the stage of the disease. Diffuse large B cell lymphoma is associated with a 5 year survival rate ranging from 70% to more than 90% among children.
Diagnosis
Staging
According to the Lugano classification, there are four stages of diffuse large B cell lymphoma based on the number of nodes involved and extranodal status.
History and symptoms
The most common symptoms of diffuse large B cell lymphoma include a rapidly enlarging, non-painful mass that may be located in neck, groin or abdomen. The less common symptoms of diffuse large B cell lymphoma include fever, weight loss and drenching night sweats.
Physical examination
Common physical examination findings of diffuse large B cell lymphoma include a non tender mass which can arise in virtually any part of the body.
Laboratory Findings
Laboratory tests of diffuse large B cell lymphoma include cytology, genetic testing, histologic confirmation, and immunophenotyping.
Electrocardiogram
There are no electrocardiogam findings associated with diffuse large B cell lymphoma.
Chest X ray
There are no chest X ray findings associated with diffuse large B cell lymphoma.
CT
CT scan may be helpful in the diagnosis of diffuse large B cell lymphoma.
Biopsy
Lymph node or extranodal tissue biopsy is diagnostic of large B cell lymphoma.
Treatment
Medical therapy
The optimal therapy for diffuse large B cell lymphoma depends on the stage at diagnosis. The predominant therapy for diffuse large B cell lymphoma is chemotherapy. Adjunctive radiotherapy may be required.
Future or Investigational therapies
Active areas of research include genetic studies to assess the genetic role in diffuse large B cell lymphoma and novel therapeutic regimens that evaluate the efficacy and safety of novel regimens in in newly diagnosed diffuse large B cell lymphoma.
References
- ↑ 1.0 1.1 "A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project". Blood. 89 (11): 3909–18. 1997. PMID 9166827.
- ↑ 2.0 2.1 Morton, L. M.; Wang, S. S.; Devesa, S. S.; Hartge, P; Weisenburger, D. D.; Linet, M. S. (2006). "Lymphoma incidence patterns by WHO subtype in the United States, 1992-2001". Blood. 107 (1): 265–76. doi:10.1182/blood-2005-06-2508. PMC 1895348. PMID 16150940.
- ↑ 3.0 3.1 3.2 3.3 Smith, A; Howell, D; Patmore, R; Jack, A; Roman, E (2011). "Incidence of haematological malignancy by sub-type: A report from the Haematological Malignancy Research Network". British Journal of Cancer. 105 (11): 1684–92. doi:10.1038/bjc.2011.450. PMC 3242607. PMID 22045184.
- ↑ 4.0 4.1 Smith, Alexandra; Roman, Eve; Howell, Debra; Jones, Richard; Patmore, Russell; Jack, Andrew; Haematological Malignancy Research Network (2010). "The Haematological Malignancy Research Network (HMRN): A new information strategy for population based epidemiology and health service research". British Journal of Haematology. 148 (5): 739–53. doi:10.1111/j.1365-2141.2009.08010.x. PMC 3066245. PMID 19958356.