| Diffuse large B cell lymphoma | |
|---|---|
| Classification and external resources | |
| ICD-O: | M9680/3 |
| MeSH | D016403 |
Diffuse large B-cell lymphoma (DLBL, DLBCL, or DLCL) is a type of aggressive lymphoma. It accounts for approximately 40% of lymphomas among adults.[1] Average age for diagnoses is between the ages of 25 and 40 years, however this type of cancer may occur at any age. It is twice as common in women as in men. Of all cancers involving the same class of blood cell, 31% of cases are DLBL.[2]
Contents |
Classification
Two[3] or three[4] major subtypes of DLBL have been identified based on their genetic activity:
- activated (ABC-DLBCL), with a pattern of genetic expression that is similar to healthy, activated B cells, and
- germinal center (GCB-DLBCL), with a pattern of genetic expression that is similar to germinal center B cells and a chromosomal translocation involving the gene bcl-2. This type has a relatively favorable prognosis.[5]
- primary mediastinal B-cell lymphoma (PMBL) Where the cancer is found in the thymus gland and lymph nodes behind the sternum (mediastinum), the area between the lungs, in the middle of the chest.
Chromosome anomalies are:[6]
- t(14;18)(q32;q21) with BCL2-rearrangement.
- t(3;Var)(q27;Var) with BCL6-rearrangement. These are common translocations.[7]
- t(8;14)(q24;q32) with MYC rearrangements.
- and other less characteristic anomalies.
Treatment
Standard treatment is CHOP. An expanded protocol, called CHOP-R, has improved survival[8] and rates of complete responses for DLBL patients, particularly elderly patients.[3] R-CHOP is a combination of one monoclonal antibody, 3 chemotherapy drugs, and one steroid: Rituximab (Rituxan®), Cyclophosphamide (Cytoxan®) Doxorubicin (Hydroxdaunorubicin®), Vincristine (Oncovin®), and Prednisone. Chemotherapy is most often administered intravenously and is most effective when it is administered multiple times over a period of months (eg. every 3 weeks, over 8-10 months). For someone who will be receiving chemotherapy it is suggested they have a PICC (Peripherally inserted central catheter) in their arm near the elbow, and the catheter will extend through one of the large veins to within centimeters of the heart. Radiation is another common treatment and is most effective if done prior to the chemotherapy, or as the last treatment after chemotherapy has been completed.
Prognosis
The germinal center subtype has the best prognosis, with 60% of treated patients surviving more than five years.[3]
References
- ^ [1] Pathobiology of the aggressive and highly aggressive non-Hodgkin's lymphomas
- ^ Turgeon, Mary Louise (2005). Clinical hematology: theory and procedures. Hagerstown, MD: Lippincott Williams & Wilkins. p. 283. ISBN 0-7817-5007-5. "Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 121.)"
- ^ a b c Turgeon, Mary Louise (2005). Clinical hematology: theory and procedures. Hagerstown, MD: Lippincott Williams & Wilkins. pp. 285–286. ISBN 0-7817-5007-5.
- ^ Lenz G, Wright GW, Emre NC, et al. (September 2008). "Molecular subtypes of diffuse large B-cell lymphoma arise by distinct genetic pathways". Proc. Natl. Acad. Sci. U.S.A. 105 (36): 13520–5. doi:. PMID 18765795. http://www.pnas.org/cgi/pmidlookup?view=long&pmid=18765795.
- ^ Shiozawa E, Yamochi-Onizuka T, Takimoto M, Ota H (November 2007). "The GCB subtype of diffuse large B-cell lymphoma is less frequent in Asian countries". Leuk. Res. 31 (11): 1579–83. doi:. PMID 17448534. http://linkinghub.elsevier.com/retrieve/pii/S0145-2126(07)00107-5.
- ^ "Diffuse large cell lymphoma" at Atlas of Genetics and Cytogenetics in Oncology and Haematology
- ^ Bosga-Bouwer AG, Haralambieva E, Booman M, et al. (November 2005). "BCL6 alternative translocation breakpoint cluster region associated with follicular lymphoma grade 3B". Genes Chromosomes Cancer 44 (3): 301–4. doi:. PMID 16075463. http://dx.doi.org/10.1002/gcc.20246.
- ^ Sehn LH, Berry B, Chhanabhai M, et al. (March 2007). "The revised International Prognostic Index (R-IPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R-CHOP". Blood 109 (5): 1857–61. doi:. PMID 17105812. http://www.bloodjournal.org/cgi/pmidlookup?view=long&pmid=17105812.
|
|||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| This article about a neoplasm is a stub. You can help Wikipedia by expanding it. |
This entry is from Wikipedia, the leading user-contributed encyclopedia. It may not have been reviewed by professional editors (see full disclaimer)
