Primary mediastinal B-cell lymphoma, abbreviated PMBL or PMBCL, is a rare type of lymphoma that forms in the mediastinum (the space in between the lungs) and predominantly affects young adults.[1][2]
| Primary mediastinal large B-cell lymphoma | |
|---|---|
 | |
| Micrograph of a primary mediastinal large B-cell lymphoma. H&E stain. | |
| Specialty | Hematology and oncology |
While it had previously been considered a subtype of diffuse large B-cell lymphoma;[3], the World Health Organization no longer classifies it as such, based on distinct clinicopathologic and molecular features.[4].
Diagnosis
editDiagnosis requires a biopsy, so that the exact type of tissue can be determined by examination under a microscope. PMBCL is a distinct type of lymphoma that shares some features with both diffuse large B-cell lymphoma, and nodular sclerosing Hodgkin lymphoma (NSHL).[5] Tumors that are even more closely related to NSHL than typical for PMBCL are called gray zone lymphoma.[5]
Treatment
editTreatment commonly begins with months of multi-drug chemotherapy regimen. Either R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) or DA-EPOCH-R (dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab) has been typical.[6] Other, more intense, regimens may be more effective.[5]
Radiation therapy may be added, especially if chemotherapy does not seem sufficient on its own.[6] Radiation may cause other health problems later, such as breast cancer, and there is some debate about the best approach to it.[5]
FDG-PET scanning is not as useful for predicting treatment success in PMBCL as it is in other lymphomas.[5]
Prognosis
editMost people with PMBCL are successfully treated and survive for many years. However, if the initial treatment is unsuccessful, or if it returns, the long-term prognosis is worse.[6] Relapses generally appear within 12 to 18 months after the completion of treatment.[5]
Epidemiology
editThis lymphoma is most commonly seen in women between the age of 20 and 40.[5]
See also
editReferences
edit- ^ Johnson PW, Davies AJ (2008). "Primary mediastinal B-cell lymphoma". Hematology. American Society of Hematology. Education Program. 2008: 349–58. doi:10.1182/asheducation-2008.1.349. PMID 19074109.
- ^ Coso D, Rey J, Bouabdallah R (February 2010). "[Primary mediastinal B-cell lymphoma]". Revue de Pneumologie Clinique. 66 (1): 32–5. doi:10.1016/j.pneumo.2009.12.007. PMID 20207294.
- ^ "Types of Non-Hodgkin Lymphoma". www.cancer.org. Retrieved 2017-09-28.
- ^ "Primary mediastinal large B-cell lymphoma". ashpublications.org/blood/article/140/9/955/476814/Primary-mediastinal-large-B-cell-lymphoma.
- ^ a b c d e f g Dunleavy K, Wilson WH (January 2015). "Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach?". Blood. 125 (1): 33–9. doi:10.1182/blood-2014-05-575092. PMC 4281829. PMID 25499450.
- ^ a b c Lees C, Keane C, Gandhi MK, Gunawardana J (February 2019). "Biology and therapy of primary mediastinal B-cell lymphoma: current status and future directions". British Journal of Haematology. 185 (1): 25–41. doi:10.1111/bjh.15778. PMC 6594147. PMID 30740662.