Primary Mediastinal (Thymic) Large 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 (thymic) large B-cell lymphoma is a distinct type of diffuse large B-cell lymphoma involving the mediastinum, recognized in the WHO 2008 classification.^:370–374

Signs and symptoms

Superior vena cava syndrome occurs in 30–50%, and pleural or pericardial effusions occur in about one-third.

Pathophysiology

PMLBCL arises from a putative thymic peripheral B cell. It has several distinctive biological features. Molecular analysis shows that PMLBCL is distinct from other types of diffuse large B-cell lymphomas (DLBCL). MAL gene expression is seen in 70%, unlike other diffuse large B-cell lymphomas.^:370 Gene expression profiling shows considerable variance from other DLBCLs and similarity to Hodgkin disease.^:290–293

PMLBCL is CD20 positive, expresses pan-B markers including CD79a, and has clonal immunoglobulin gene rearrangements and mRNA but paradoxically does not express cytoplasmic or cell surface immunoglobulin.^:370

Clinically, PMLBCL is unusual in several respects. Despite 80% PMLBCL being stage I or II, the presenting anterior mediastinal mass is often over 10 cm and is locally invasive of lung, chest wall, pleura, and pericardium. At initial presentation, PMLBCL is usually confined to mediastinum, but its bulk, rather than additional adenopathy, can sometimes be palpated at the low neck. Increased LDH is seen in approximately 75%,^:370 but unlike other large cell lymphomas, no increase in beta-2 microglobulin is seen even when bulky^:370 which may relate to defective major histocompatibility complex expression.^:370

Treatment

Multiagent chemotherapy is recommended, but the preferred regimen is controversial, as is consolidative radiotherapy.

Epidemiology

It affects primarily young adults; the median age is 37 years.^:370 It is more common in females.

Grey zone lymphoma