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Medical condition From Wikipedia, the free encyclopedia
Primary mediastinal B-cell lymphoma, abbreviated PMBL, 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 | |
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Micrograph of a primary mediastinal large B-cell lymphoma. H&E stain. | |
Specialty | Hematology and oncology |
It is a subtype of diffuse large B-cell lymphoma;[3] however, it generally has a significantly better prognosis.
Diagnosis requires a biopsy, so that the exact type of tissue can be determined by examination under a microscope. PMBL is generally considered a sub-type of diffuse large B-cell lymphoma, although it is also closely related to nodular sclerosing Hodgkin lymphoma (NSHL).[4] Tumors that are even more closely related to NSHL than typical for PMBL are called gray zone lymphoma.[4]
Treatment 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.[5] Other, more intense, regimens may be more effective.[4]
Radiation therapy may be added, especially if chemotherapy does not seem sufficient on its own.[5] Radiation may cause other health problems later, such as breast cancer, and there is some debate about the best approach to it.[4]
FDG-PET scanning is not as useful for predicting treatment success in PMBL as it is in other lymphomas.[4]
Most people with PMBL 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.[5] Relapses generally appear within 12 to 18 months after the completion of treatment.[4]
This lymphoma is most commonly seen in women between the age of 20 and 40.[4]
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