primary mediastinal large B-cell lymphoma

Summary

primary mediastinal large B-cell lymphoma (MONDO:0020323) is a cancer and 102 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, epcoritamab, and axicabtagene ciloleucel. A subtype of thymus lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: 1-9 / 100 000 (Europe) [Orphanet-validated]
• Clinical trials: 102

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Point prevalence	1-9 / 100 000	5	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	primary mediastinal large B-cell lymphoma
Mondo ID	MONDO:0020323
Orphanet	98838
DOID	DOID:0080210
ICD-10-CM	C85.2
ICD-11	950282079
NCIT	C9280
SNOMED CT	444910004
UMLS	C1292754
MedGen	714984
GARD	0019589
MedDRA	10036710
Is cancer (heuristic)	yes

Also known as: B-cell diffuse large cell lymphoma of mediastinum · B-cell diffuse large cell lymphoma of the mediastinum · large cell lymphoma of the mediastinum · Med-DLBCL · mediastinal (thymic) large B-cell lymphoma · mediastinal B-cell diffuse large cell lymphoma · mediastinal diffuse large cell lymphoma with sclerosis · mediastinal diffuse large-cell lymphoma with sclerosis · mediastinal large B-cell lymphoma · PMBL · PMLCL · primary mediastinal (thymic) large B-cell lymphoma · primary mediastinal clear cell lymphoma of B-cell type · primary mediastinal large B-cell lymphoma

Data availability: 3 cell lines.

Disease family

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › immune system cancer › thymus cancer › thymus lymphoma › primary mediastinal large B-cell lymphoma

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 102.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	34
PHASE1	26
PHASE1/PHASE2	20
Not specified	19
EARLY_PHASE1	2
PHASE3	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT04759586	PHASE3	ACTIVE_NOT_RECRUITING	Nivolumab in Combination With Chemo-Immunotherapy for the Treatment of Newly Diagnosed Primary Mediastinal B-Cell Lymphoma
NCT02628405	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	R-ICE and Lenalidomide in Treating Patients With First-Relapse/Primary Refractory Diffuse Large B-Cell Lymphoma
NCT03038672	PHASE2	ACTIVE_NOT_RECRUITING	Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas
NCT03625037	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	First-in-Human (FIH) Trial in Patients With Relapsed, Progressive or Refractory B-Cell Lymphoma
NCT04186520	PHASE1/PHASE2	RECRUITING	CAR-20/19-T Cells in Patients With Relapsed Refractory B Cell Malignancies
NCT04257578	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Acalabrutinib and Anti-CD19 CAR T-cell Therapy for the Treatment of B-cell Lymphoma
NCT04542824	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Trial of the Safety and Efficacy of Epcoritamab in Japanese Subjects With Relapsed or Refractory (R/R) B-Cell Non-Hodgkin Lymphoma (R/R B-NHL)
NCT04745559	PHASE2	ACTIVE_NOT_RECRUITING	Optimizing Cellular and Humoral Immunity by Vaccinating With PCV13 Before and After CAR-T Therapy
NCT04836507	PHASE1/PHASE2	RECRUITING	Study of Efficacy and Safety of CRC01 in Adult Large B-cell Lymphoma Patients
NCT05025800	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	ALX148, Rituximab and Lenalidomide for the Treatment of Indolent and Aggressive B-cell Non-Hodgkin Lymphoma
NCT05202782	PHASE2	ACTIVE_NOT_RECRUITING	Zanubrutinib and CAR T-cell Therapy for the Treatment of Recurrent or Refractory Aggressive B-cell Non-Hodgkin’s Lymphoma or Transformed Indolent B-cell Lymphoma
NCT05326243	PHASE1/PHASE2	RECRUITING	Phase 1/2 Study of CD19 Chimeric Antigen Receptor T-cell (CD19 CAR-T; PL001) for Relapsed or Refractory B-cell Lymphoma
NCT05507541	PHASE2	ACTIVE_NOT_RECRUITING	TTI-622 in Combination With Pembrolizumab for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma
NCT05633615	PHASE2	RECRUITING	Testing Drug Treatments After CAR T-cell Therapy in Patients With Relapsed/Refractory Diffuse Large B-cell Lymphoma
NCT05672251	PHASE2	RECRUITING	Loncastuximab Tesirine and Mosunetuzumab for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma
NCT05821088	PHASE2	RECRUITING	Tafasitamab and Lenalidomide Followed by Tafasitamab and ICE as Salvage Therapy for Transplant Eligible Patients With Relapsed/ Refractory Large B-Cell Lymphoma
NCT05934448	PHASE2	RECRUITING	Pembro Plus CAR T-cell Therapy in R/R in PMBCL
NCT06093841	PHASE2	ACTIVE_NOT_RECRUITING	Relmacabtagene Autoleucel As Second-Line Therapy in Adult Patients with Aggressive B-cell NHL
NCT06238648	PHASE2	RECRUITING	Epcoritamab Compared to Observation for Treating B-cell Lymphoma Patients Not in Complete Remission After CD19-directed CAR-T Therapy
NCT06271057	PHASE2	RECRUITING	Golcadomide Post-CAR T-cell in R/R Aggressive Large B-cell Lymphoma Patients With High Risk of Relapse
NCT06412068	PHASE2	NOT_YET_RECRUITING	A Prospective Study: Sintilimab and R-CHOP in PMBCL Treatment
NCT06486051	PHASE2	RECRUITING	A Study of WZTL-002 CAR T-cells for Adults With Relapsed Large B-cell Lymphoma
NCT06504394	PHASE2	ACTIVE_NOT_RECRUITING	A Study to Evaluate the Safety and Efficacy of Pembrolizumab (MK-3475) Coformulated With Berahyaluronidase Alfa (MK-3475A) in Participants With Relapsed or Refractory Classical Hodgkin Lymphoma (rrcHL) or Relapsed or Refractory Primary Mediastinal Large B-cell Lymphoma (rrPMBCL)(MK-3475A-F65)
NCT06533579	PHASE1/PHASE2	RECRUITING	Gene Therapy for CD19-Positive Hematologic Malignancies (SENTRY-CD19)
NCT06536049	PHASE1/PHASE2	RECRUITING	Epcoritamab Plus Ibrutinib for the Treatment of Relapsed or Refractory Aggressive B-Cell Non-Hodgkin Lymphoma
NCT06550141	PHASE2	RECRUITING	Emapalumab Prevention of CAR-T Cell Associated Toxicities
NCT06784726	PHASE2	RECRUITING	Odronextamab for Relapsed and Refractory Large B-cell Lymphomas Before CAR-T
NCT06834373	PHASE2	RECRUITING	Golcadomide and Rituximab as Bridging Therapy for Relapsed or Refractory Aggressive B-cell Non-Hodgkin Lymphoma Before CAR T-cell Therapy
NCT06871007	PHASE1/PHASE2	ENROLLING_BY_INVITATION	Nivolumab and DA-EPOCH-R in Pediatric Primary Mediastinal (Thymic) Large B-cell Lymphoma
NCT07097363	PHASE2	RECRUITING	Epcoritamab With Dose Adjusted Etoposide, Cyclophosphamide, Vincristine, Doxorubicin, Prednisone and Rituximab (EPOCH-R) for the Treatment of Aggressive B-Cell Non-Hodgkin Lymphoma
NCT07098364	PHASE1/PHASE2	RECRUITING	ST-067 in Combination With CD19-Directed CAR T-Cell Therapy (Liso-cel) in Relapsed/Refractory Large B-Cell Lymphoma
NCT07365306	PHASE2	RECRUITING	Epcoritamab, Rituximab, Gemcitabine and Oxaliplatin (R-GemOx) as Salvage Therapy Before Autologous Stem Cell Transplant for the Treatment of Relapsed/Refractory Diffuse Large B-Cell Lymphoma
NCT07473167	PHASE1/PHASE2	RECRUITING	Study of Safety and Efficacy of TC011 in the Relapsed/Refractory Large B Cell Non-Hodgkin Lymphoma Patients
NCT00001337	PHASE2	COMPLETED	Dose-Adjusted EPOCH Chemotherapy and Rituximab (CD20+) in Previously Untreated Aggressive Non-Hodgkin’s Lymphoma
NCT00532259	PHASE2	COMPLETED	CT-011 MAb in DLBCL Patients Following ASCT
NCT01516567	PHASE2	COMPLETED	Intergroup Trial for Children or Adolescents With Primary Mediastinal Large B-Cell Lymphoma: DA-EPOCH-Rituximab Evaluation
NCT01897571	PHASE1/PHASE2	COMPLETED	Study of Tazemetostat as Single Agent in Solid Tumors or B-cell Lymphomas and in Combination With Prednisolone in DLBCL
NCT01965119	PHASE2	COMPLETED	Pilot Study of Ruxolitinib in Relapsed or Refractory Hodgkin Lymphoma and Primary Mediastinal Large B-cell Lymphoma
NCT02362035	PHASE1/PHASE2	COMPLETED	ACP-196 (Acalabrutinib) in Combination With Pembrolizumab, for Treatment of Hematologic Malignancies
NCT02423291	PHASE2	COMPLETED	A Study of SGN-35 (Brentuximab Vedotin) of Patients With Relapsed or Refractory PMLBCL

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
CYCLOPHOSPHAMIDE ANHYDROUS	4	19
EPCORITAMAB	4	8
AXICABTAGENE CILOLEUCEL	4	5
PROCHLORPERAZINE	4	3
ACALABRUTINIB	4	2
ANAKINRA	4	1
BRENTUXIMAB VEDOTIN	4	1
COPANLISIB HYDROCHLORIDE	4	1
DOCUSATE	4	1
ETOPOSIDE PHOSPHATE	4	1
LACTULOSE	4	1
OMEPRAZOLE	4	1
PEGFILGRASTIM	4	1
TAZEMETOSTAT	4	1
CAMRELIZUMAB	3	1
PIDILIZUMAB	2	1
VARLILUMAB	2	1
CHEMBL4747506	0	2
CHEMBL5187554	0	2
CHEMBL5276925	0	2
CHEMBL5398431	0	1
CHEMBL4637163	0	1
CHEMBL5220618	0	1