intravascular large B-cell lymphoma

Summary

intravascular large B-cell lymphoma (MONDO:0020324) is a cancer and 9 clinical trials. Top therapeutic interventions include cyclophosphamide anhydrous, zanubrutinib, and azacitidine. 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: 9

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	intravascular large B-cell lymphoma
Mondo ID	MONDO:0020324
Orphanet	98839
DOID	DOID:0081311
ICD-11	952730197
NCIT	C4342
SNOMED CT	255102004
UMLS	C0334660
MedGen	90821
GARD	0019590
MedDRA	10069643
Is cancer (heuristic)	yes

Also known as: angioendotheliomatosis proliferans systemisata · angiotropic large cell lymphoma · angiotropic lymphoma · intravascular B-cell lymphoma · intravascular large B-cell lymphoma · intravascular lymphomatosis · IVBCL · malignant angioendotheliomatosis · Tappeiner-Pfleger disease

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 › intravascular large B-cell lymphoma

Related subtypes (29): relapsed/refractory diffuse large B-cell lymphoma, breast diffuse large B-cell lymphoma, colorectal diffuse large B-cell lymphoma, gastric diffuse large B-cell lymphoma, liver diffuse large B-cell lymphoma, primary cutaneous diffuse large B-cell lymphoma, Leg type, primary pulmonary diffuse large B-cell lymphoma, small intestinal diffuse large B-cell lymphoma, splenic diffuse large B-cell lymphoma, thyroid gland diffuse large B-cell lymphoma, Epstein-Barr virus-positive diffuse large B-cell lymphoma of the elderly, plasmablastic lymphoma, diffuse large B-cell lymphoma of the central nervous system, T-cell/histiocyte rich large B cell lymphoma, diffuse large B-cell lymphoma with chronic inflammation, ALK-positive large B-cell lymphoma, primary effusion lymphoma, lymphomatoid granulomatosis, primary mediastinal large B-cell lymphoma, high grade B-cell lymphoma, diffuse large B-cell lymphoma activated B-cell type, diffuse large B-cell lymphoma germinal center B-cell type, BN2 diffuse large B-cell lymphoma, EZB diffuse large B-cell lymphoma, MCD diffuse large B-cell lymphoma, N1 diffuse large B-cell lymphoma, ST2 diffuse large B-cell lymphoma, A53 diffuse large B-cell lymphoma, primary vitreoretinal 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: 9.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	6
PHASE2/PHASE3	1
PHASE1/PHASE2	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT04799275	PHASE2/PHASE3	ACTIVE_NOT_RECRUITING	Testing CC-486 (Oral Azacitidine) Plus the Standard Drug Therapy in Patients 75 Years or Older With Newly Diagnosed 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
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
NCT05507541	PHASE2	ACTIVE_NOT_RECRUITING	TTI-622 in Combination With Pembrolizumab for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma
NCT06736613	PHASE2	RECRUITING	A Study of Circulating Tumor DNA (ctDNA) Testing for People With B-Cell Lymphoma
NCT06834373	PHASE2	RECRUITING	Golcadomide and Rituximab as Bridging Therapy for Relapsed or Refractory Aggressive B-cell Non-Hodgkin Lymphoma Before CAR T-cell Therapy
NCT03704714	PHASE1/PHASE2	SUSPENDED	Nivolumab and Combination Chemotherapy in Treating Participants With Diffuse Large B-Cell Lymphoma
NCT04899570	PHASE2	UNKNOWN	Zanubrutinib Combined With R-CHOP in Newly-diagnosed Intravascular Large B-cell Lymphoma
NCT05907447	Not specified	UNKNOWN	A Retrospective Study on Extranodal Lymphoma or Lymphoma of Rare Pathological Types

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
CYCLOPHOSPHAMIDE ANHYDROUS	4	3
ZANUBRUTINIB	4	2
AZACITIDINE	4	1
VINDESINE	4	1
GOLCADOMIDE	3	1
MAPLIRPACEPT	2	1
ONTORPACEPT	2	1
VARLILUMAB	2	1
CHEMBL5193128	0	2