词条 | Primary mediastinal B-cell lymphoma |
释义 |
| name = Primary mediastinal large B-cell lymphoma | synonyms = | image = Primary mediastinal large B-cell lymphoma - very high mag.jpg | caption = Micrograph of a primary mediastinal large B-cell lymphoma. H&E stain. | pronounce = | field = Hematology and oncology | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}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] It is a subtype of diffuse large B-cell lymphoma;[3] however, it generally has a significantly better prognosis. DiagnosisDiagnosis 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] TreatmentTreatment 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]PrognosisMost 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] EpidemiologyThis lymphoma is most commonly seen in women between the age of 20 and 40.[4] See also
References1. ^{{cite journal | vauthors = Johnson PW, Davies AJ | title = Primary mediastinal B-cell lymphoma | journal = Hematology. American Society of Hematology. Education Program | volume = 2008 | issue = | pages = 349–58 | year = 2008 | pmid = 19074109 | doi = 10.1182/asheducation-2008.1.349 }} 2. ^{{cite journal | vauthors = Coso D, Rey J, Bouabdallah R | title = [Primary mediastinal B-cell lymphoma] | journal = Revue De Pneumologie Clinique | volume = 66 | issue = 1 | pages = 32–5 | date = February 2010 | pmid = 20207294 | doi = 10.1016/j.pneumo.2009.12.007 }} 3. ^{{Cite web|url=https://www.cancer.org/cancer/non-hodgkin-lymphoma/about/types-of-non-hodgkin-lymphoma.html|title=Types of Non-Hodgkin Lymphoma|website=www.cancer.org|access-date=2017-09-28}} 4. ^1 2 3 4 5 6 {{cite journal | vauthors = Dunleavy K, Wilson WH | title = Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: do they require a unique therapeutic approach? | journal = Blood | volume = 125 | issue = 1 | pages = 33–9 | date = January 2015 | pmid = 25499450 | pmc = 4281829 | doi = 10.1182/blood-2014-05-575092 }} 5. ^1 2 {{cite journal | vauthors = Lees C, Keane C, Gandhi MK, Gunawardana J | title = Biology and therapy of primary mediastinal B-cell lymphoma: current status and future directions | journal = British Journal of Haematology | date = February 2019 | pmid = 30740662 | doi = 10.1111/bjh.15778 }} External links{{Medical resources| DiseasesDB = | ICD10 = | ICD9 = | ICDO = 9679/3 | OMIM = | MedlinePlus = | eMedicineSubj = article | eMedicineTopic = 203681 | MeshID = }}{{Lymphoid malignancy}} 1 : Lymphoma |
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