enteropathy-associated T-cell lymphoma

Summary

enteropathy-associated T-cell lymphoma (MONDO:0019473) is a cancer and 26 clinical trials. Top therapeutic interventions include brentuximab vedotin, cyclophosphamide anhydrous, and doxorubicin. A subtype of T-cell non-Hodgkin lymphoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: <1 / 1 000 000 (United States) [Orphanet-validated]
• Clinical trials: 26

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	<1 / 1 000 000	0.0111	United States	Validated
Annual incidence	1-9 / 1 000 000	0.1	Netherlands	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	enteropathy-associated T-cell lymphoma
Mondo ID	MONDO:0019473
MeSH	D058527
Orphanet	86880
ICD-10-CM	C86.2
ICD-11	958629729
NCIT	C4737
SNOMED CT	277654008
UMLS	C0456889
MedGen	96832
GARD	0009809
MedDRA	10022703
Is cancer (heuristic)	yes

Also known as: EATCL · EATL · EATL, type I · enteropathy associated T-cell lymphoma · enteropathy-associated T-cell lymphoma, type I · enteropathy-type T-cell lymphoma · ETTL · high-grade pleomorphic peripheral T-cell lymphoma · intestinal T-cell lymphoma

Disease family

This is a subtype of T-cell non-Hodgkin 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 by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › hematopoietic and lymphoid system neoplasm › hematopoietic and lymphoid cell neoplasm › lymphoid neoplasm › lymphoma › non-Hodgkin lymphoma › T-cell non-Hodgkin lymphoma › enteropathy-associated T-cell lymphoma

Related subtypes (8): mature T-cell and NK-cell non-Hodgkin lymphoma, primary cutaneous T-cell lymphoma, CD4+/CD56+ hematodermic neoplasm, adult T-cell leukemia/lymphoma, hepatosplenic T-cell lymphoma, T-lymphoblastic lymphoma, Nodal T-follicular helper cell lymphoma, follicular type, monomorphic epitheliotropic intestinal T-cell lymphoma

Subtypes (1): small intestinal enteropathy-associated T-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

Drugs indicated or in trials for this disease

No drug has an approved disease-direct ChEMBL indication for this disease.

1 drug in clinical trials for this disease (phase 2–3, investigational): efficacy not established — a trial record, not an indication.

Drug	Highest phase
Brentuximab Vedotin	Phase 2

Clinical trials & evidence

Clinical trials

Clinical trials: 26.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	11
PHASE1	7
PHASE1/PHASE2	4
Not specified	3
PHASE4	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01746992	PHASE4	UNKNOWN	CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for Newly Diagnosed Young Patients With T Cell Lymphoma
NCT03113500	PHASE2	ACTIVE_NOT_RECRUITING	Brentuximab Vedotin and Combination Chemotherapy in Treating Patients With CD30-Positive Peripheral T-cell Lymphoma
NCT03598998	PHASE1/PHASE2	ACTIVE_NOT_RECRUITING	Pembrolizumab and Pralatrexate in Treating Patients With Relapsed or Refractory Peripheral T-Cell Lymphomas
NCT05475925	PHASE1/PHASE2	RECRUITING	A Study of DR-01 in Subjects With Large Granular Lymphocytic Leukemia or Cytotoxic Lymphomas
NCT06701344	PHASE2	NOT_YET_RECRUITING	Go-CHOP in de Novo Intestinal T-cell Lymphoma Patients
NCT00901147	PHASE2	COMPLETED	Study of Bortezomib and Panobinostat in Treating Patients With Relapsed/Refractory Peripheral T-cell Lymphoma or NK/T-cell Lymphoma
NCT01719835	PHASE2	UNKNOWN	CHOP vs GEM-P in 1st Line Treatment of T-cell Lymphoma, Multicentre Phase II Study
NCT02533700	PHASE2	UNKNOWN	CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL
NCT02561273	PHASE1/PHASE2	COMPLETED	Combination Chemotherapy & Lenalidomide in Newly Diagnosed Stage II-IV Peripheral T-cell Non-Hodgkin’s Lymphoma
NCT02588651	PHASE2	COMPLETED	A Phase II Study of Single Agent Brentuximab Vedotin in Relapsed/Refractory CD30 Low (<10%) Mature T Cell Lymphoma (TCL)
NCT03075553	PHASE2	TERMINATED	Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma
NCT03217643	PHASE2	COMPLETED	CHP-BV Followed by Consolidation With High-dose Therapy / ASCT as Frontline Treatment of Patients With EATL Type 1.
NCT04423926	PHASE1/PHASE2	UNKNOWN	Lenalidomide in Combination With CHOP in Patients With Untreated PTCL
NCT04795869	PHASE2	WITHDRAWN	Brentuximab Vedotin and Pembrolizumab in Treating Patients With Recurrent Peripheral T-Cell Lymphoma
NCT04803201	PHASE2	SUSPENDED	Testing the Addition of Duvelisib or CC-486 to the Usual Treatment for Peripheral T-Cell Lymphoma
NCT06692452	PHASE2	WITHDRAWN	Tazemetostat Plus CHOP in 1L T-cell Lymphoma
NCT05377827	PHASE1	ACTIVE_NOT_RECRUITING	Dose-Escalation and Dose-Expansion Study to Evaluate the Safety and Tolerability of Anti-CD7 Allogeneic CAR T-Cells (WU-CART-007) in Patients With CD7+ Hematologic Malignancies
NCT07055477	PHASE1	RECRUITING	A Phase I Trial Anti-CC Chemokine Receptor 4 Chimeric Antigen Receptor T Cells (CCR4 CAR T Cells) for CCR4 Expressing T-cell Malignancies Including Peripheral T-cell Non-Hodgkin Lymphoma (PTCL) and Cutaneous T-cell Non-Hodgkin Lymphoma (CTCL)
NCT00697346	PHASE1	COMPLETED	Study of MLN8237 in Participants With Advanced Hematological Malignancies
NCT01590732	PHASE1	COMPLETED	Romidepsin, Ifosfamide, Carboplatin, and Etoposide in Treating Participants With Relapsed or Refractory Peripheral T-Cell Lymphoma
NCT03049449	PHASE1	COMPLETED	T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas
NCT04480788	PHASE1	UNKNOWN	CD7-CART in the Treatment of r / r CD7 Positive Hemolymph System Malignancies on Increasing Dose and Open Label Study
NCT04639843	PHASE1	WITHDRAWN	Doxorubicin, CC-(486) (5-azacitidine), Romidepsin, and Duvelisib (hARD) for T-cell Lymphoma
NCT01787409	Not specified	ACTIVE_NOT_RECRUITING	Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency
NCT05978141	Not specified	RECRUITING	A Registry for People With T-cell Lymphoma
NCT02652715	Not specified	COMPLETED	Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
BRENTUXIMAB VEDOTIN	4	3
CYCLOPHOSPHAMIDE ANHYDROUS	4	3
DOXORUBICIN	4	3
DUVELISIB	4	2
IFOSFAMIDE	4	2
ROMIDEPSIN	4	2
ETOPOSIDE	4	1
ETOPOSIDE PHOSPHATE	4	1
METHOTREXATE	4	1
PANOBINOSTAT	4	1
PRALATREXATE	4	1
PREDNISONE	4	1
TAZEMETOSTAT	4	1
VINCRISTINE	4	1
ALISERTIB	3	1
PIRARUBICIN	3	1
DIBOTATUG	2	1
GOLIDOCITINIB	2	1
CHEMBL3805348	0	2
CHEMBL4538684	0	2
CHEMBL4066465	0	2
CHEMBL15720	0	1
CHEMBL426	0	1
CHEMBL344227	0	1
CHEMBL5398431	0	1