Primary Mediastinal B-Cell Lymphoma

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Retrieved

2021-01-18

Source

Wikipedia

Trials

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Genes

XPO1, BCL6, MAL, JAK2, CD274, MYC, MS4A1, PDCD1LG2, KRT20, SOCS1, CIITA, PAX5, FSCN1, IL4, BCL2, GATA3, FOXP1, CGB8, REL, RPE65, SLC22A2, SOAT1, STAT6, TNFAIP3, IL4I1, UVRAG, CGB5, CHRDL2, TCL1A, TP63, CARD11, AICDA, CD83, PIK3CG, TCL1B, CKAP4, POU2F2, AKT1, PIK3CD, PIK3CB, BCL2A1, CD19, TNFRSF8, CGA, CGB3, EZH2, FCER2, FUT4, HTC2, IGH, JCHAIN, IL3RA, IL4R, IL13, MAOA, BORCS8-MEF2B, KMT2A, CD200, NFKB1, NFKBIE, NOS1, NOS2, CCND1, PDCD1, PIK3CA, MEF2B

Drugs

3-(5-amino-2-methyl-4-oxoquinazolin-3(4H)-yl)piperidine-2,6-dione hydrochloride, 5-(4,6-dimorpholino-1,3,5-triazin-2-yl)-4-(trifluoromethyl)pyridin-2-amine, Allogeneic multi-virus specific T lymphocytes targeting BK virus, cytomegalovirus, human herpesvirus-6, Epstein Barr virus and adenovirus, Autologous CD4+ and CD8+ T cells expressing a CD19-specific chimeric antigen receptor, Autologous engineered anti -CD19 Chimeric Antigen Receptor (CAR +) T -cells ( KYMRIAH ), Axicabtagene ciloleucel ( YESCARTA ), Brentuximab vedotin ( ADCETRIS ), Enzastaurin hydrochloride, Everolimus ( AFINITOR, VOTUBIA ), Humanised Fc engineered monoclonal antibody against CD19, Humanised anti-CD37 monoclonal antibody conjugated to maytansinoid DM1, Ibrutinib ( IMBRUVICA ), Iodine (131I) tositumomab, Lenalidomide ( LENALIDOMIDE ACCORD, REVLIMID ), Mocetinostat, Obinutuzumab ( GAZYVA, GAZYVARO ), Pixantrone dimaleate ( PIXUVRI ), Polatuzumab vedotin ( POLIVY ), Pralatrexate ( FOLOTYN ), Recombinant histidine-tagged idiotype immunoglobulin Fab fragment of clonal B-cell receptors, Rituximab ( BLITZIMA, MABTHERA, TRUXIMA, RITEMVIA, RITUXAN, RITUZENA, RIXATHON, RIXIMYO, Ruxience ), Selinexor ( XPOVIO ), Tazemetostat ( TAZVERIK ), Valproic acid, sodium, Venetoclax ( VENCLEXTA, VENCLYXTO ), haematopoietic stem cells and blood progenitors umbilical cord-derived expanded with (1R, 4R)-N1-(2-benzyl-7-(2-methyl-2H-tetrazol-5-yl)-9H-pyrimido[4,5-b]indol-4-yl)cyclohexane-1,4-diamine dihydrobromide dihydrate

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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.

It is a subtype of diffuse large B-cell lymphoma; however, it generally has a significantly better prognosis.

Diagnosis

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). Tumors that are even more closely related to NSHL than typical for PMBL are called gray zone lymphoma.

Treatment

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. Other, more intense, regimens may be more effective.

Radiation therapy may be added, especially if chemotherapy does not seem sufficient on its own. Radiation may cause other health problems later, such as breast cancer, and there is some debate about the best approach to it.

FDG-PET scanning is not as useful for predicting treatment success in PMBL as it is in other lymphomas.

Prognosis

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. Relapses generally appear within 12 to 18 months after the completion of treatment.

Epidemiology

This lymphoma is most commonly seen in women between the age of 20 and 40.

Primary Mediastinal B-Cell Lymphoma

Diagnosis

Treatment

Prognosis

Epidemiology

See also