diffuse large B-cell lymphoma of the central nervous system

disease

On this page

Also known as central nervous system diffuse large B-cell lymphomaCNS DLBCLdiffuse large B-cell lymphoma of central nervous systemDLBCL of the CNSprimary diffuse large B-cell lymphoma of the central nervous systemprimary DLBCL of the CNS

Summary

diffuse large B-cell lymphoma of the central nervous system (MONDO:0017596) is a cancer and 4 clinical trials. Top therapeutic interventions include nivolumab and varlilumab. A subtype of diffuse large B-cell lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

Classification: Cancer
Clinical trials: 4

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	diffuse large B-cell lymphoma of the central nervous system
Mondo ID	MONDO:0017596
Orphanet	300849
DOID	DOID:0081313
ICD-11	1029172881
NCIT	C71720
SNOMED CT	734066005
UMLS	C2026186
MedGen	378339
GARD	0021246
Anatomy (UBERON)	UBERON:0001017
Is cancer (heuristic)	yes

Also known as: central nervous system diffuse large B-cell lymphoma · CNS DLBCL · diffuse large B-cell lymphoma of central nervous system · DLBCL of the CNS · primary diffuse large B-cell lymphoma of the central nervous system · primary DLBCL of the CNS

Disease family

This is a subtype of diffuse large B-cell lymphoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › immune system disorder › leukocyte disorder › B-cell neoplasm › neoplasm of mature B-cells › diffuse large B-cell lymphoma › diffuse large B-cell lymphoma of the central nervous system

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: 4.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE1	3
PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT03038672	PHASE2	ACTIVE_NOT_RECRUITING	Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas
NCT04609046	PHASE1	RECRUITING	Testing the Addition of Lenalidomide and Nivolumab to the Usual Treatment for Primary CNS Lymphoma
NCT05211336	PHASE1	ACTIVE_NOT_RECRUITING	Venetoclax, Ibrutinib, Prednisone, Obinutuzumab, and Revlimid (VIPOR) for Diffuse Large B-cell Lymphoma Involving the Central Nervous System
NCT01722305	PHASE1	COMPLETED	Pomalidomide and Dexamethasone in Treating Patients With Relapsed or Refractory Primary Central Nervous System Lymphoma or Newly Diagnosed or Relapsed or Refractory Intraocular Lymphoma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
NIVOLUMAB	4	2
VARLILUMAB	2	1

Drugs: Nivolumab