MCID: ENT008
MIFTS: 38

Enteropathy-Associated T-Cell Lymphoma

Categories: Rare diseases, Cancer diseases, Gastrointestinal diseases, Blood diseases, Immune diseases

Aliases & Classifications for Enteropathy-Associated T-Cell Lymphoma

MalaCards integrated aliases for Enteropathy-Associated T-Cell Lymphoma:

Name: Enteropathy-Associated T-Cell Lymphoma 53 59 73
High-Grade Pleomorphic Peripheral T-Cell Lymphoma 53
Enteropathy-Type T-Cell Lymphoma 59
Intestinal T-Cell Lymphoma 59
Eatcl 53
Eatl 59
Ettl 59

Characteristics:

Orphanet epidemiological data:

59

Classifications:



External Ids:

Orphanet 59 ORPHA86880
MESH via Orphanet 45 D058527
UMLS via Orphanet 74 C0456889
ICD10 via Orphanet 34 C86.2
ICD10 33 C86.2
UMLS 73 C0456889

Summaries for Enteropathy-Associated T-Cell Lymphoma

MalaCards based summary : Enteropathy-Associated T-Cell Lymphoma, also known as high-grade pleomorphic peripheral t-cell lymphoma, is related to lymphoma and celiac disease 1. An important gene associated with Enteropathy-Associated T-Cell Lymphoma is CCL11 (C-C Motif Chemokine Ligand 11), and among its related pathways/superpathways are Akt Signaling and G-protein signaling_Regulation of RAC1 activity. The drugs Gemcitabine and Etoposide have been mentioned in the context of this disorder. Affiliated tissues include t cells, small intestine and bone, and related phenotypes are hematopoietic system and immune system

Wikipedia : 76 Enteropathy-associated T-cell lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type... more...

Related Diseases for Enteropathy-Associated T-Cell Lymphoma

Diseases related to Enteropathy-Associated T-Cell Lymphoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 26)
# Related Disease Score Top Affiliating Genes
1 lymphoma 11.0
2 celiac disease 1 10.5
3 refractory celiac disease 10.3
4 b-cell lymphomas 10.1
5 mucositis 10.1
6 histiocytosis 10.1
7 malignant histiocytosis 10.1
8 type i 10.1
9 autoimmune enteropathy 10.1
10 anemia, autoimmune hemolytic 9.9
11 myocardial infarction 9.9
12 cholangitis, primary sclerosing 9.9
13 hemolytic anemia 9.9
14 hemophagocytic lymphohistiocytosis 9.9
15 peripheral t-cell lymphoma 9.9
16 ulcerative colitis 9.9
17 colitis 9.9
18 jejunoileitis 9.9
19 obstructive jaundice 9.9
20 sclerosing cholangitis 9.9
21 intestinal perforation 9.9
22 restrictive cardiomyopathy 9.9
23 reticulosarcoma 9.9
24 cholangitis 9.9
25 autoinflammation, lipodystrophy, and dermatosis syndrome 9.3 CCL11 SETD2
26 gastrointestinal allergy 9.2 CCL11 ITGAE

Graphical network of the top 20 diseases related to Enteropathy-Associated T-Cell Lymphoma:



Diseases related to Enteropathy-Associated T-Cell Lymphoma

Symptoms & Phenotypes for Enteropathy-Associated T-Cell Lymphoma

MGI Mouse Phenotypes related to Enteropathy-Associated T-Cell Lymphoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.26 CCL11 CD3G ITGAE SETD2
2 immune system MP:0005387 8.92 CCL11 CD3G ITGAE SETD2

Drugs & Therapeutics for Enteropathy-Associated T-Cell Lymphoma

Drugs for Enteropathy-Associated T-Cell Lymphoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 94)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Gemcitabine Approved Phase 4,Phase 2 95058-81-4 60750
2
Etoposide Approved Phase 4,Phase 2,Phase 1 33419-42-0 36462
3
Cisplatin Approved Phase 4,Phase 2 15663-27-1 84093 441203 2767
4
Doxorubicin Approved, Investigational Phase 4,Phase 2,Phase 1 23214-92-8 31703
5
Ifosfamide Approved Phase 4,Phase 2 3778-73-2 3690
6
Vincristine Approved, Investigational Phase 4,Phase 2,Phase 1 2068-78-2, 57-22-7 5978
7
Methotrexate Approved Phase 4,Phase 2 1959-05-2, 59-05-2 126941
8
Cyclophosphamide Approved, Investigational Phase 4,Phase 2,Phase 1 50-18-0, 6055-19-2 2907
9
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
10
Epirubicin Approved Phase 4,Phase 2 56420-45-2 41867
11
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2 53-03-2 5865
12
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
13
leucovorin Approved, Nutraceutical Phase 4,Phase 2 58-05-9 143 6006
14
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2 59-30-3 6037
15
Doxil Approved June 1999 Phase 4,Phase 2,Phase 1 31703
16
Pirarubicin Investigational Phase 4 72496-41-4
17 Dexamethasone acetate Phase 4 1177-87-3
18
Isophosphamide mustard Phase 4,Phase 2 0
19 Alkylating Agents Phase 4,Phase 2,Phase 1
20 glucocorticoids Phase 4,Phase 2,Phase 1
21 Folic Acid Antagonists Phase 4,Phase 2
22 Topoisomerase Inhibitors Phase 4,Phase 2,Phase 1
23 Hormone Antagonists Phase 4,Phase 2,Phase 1
24 Dermatologic Agents Phase 4,Phase 2,Phase 1
25 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
26 Hormones Phase 4,Phase 2,Phase 1
27 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 1
28 Anti-Bacterial Agents Phase 4,Phase 2,Phase 1
29 Vitamin B Complex Phase 4,Phase 2
30 Antibiotics, Antitubercular Phase 4,Phase 2,Phase 1
31 Etoposide phosphate Phase 4,Phase 2,Phase 1
32 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
33 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 1
34 Antirheumatic Agents Phase 4,Phase 2,Phase 1
35 BB 1101 Phase 4
36 Antimetabolites Phase 4,Phase 2
37 Antimetabolites, Antineoplastic Phase 4,Phase 2
38 Antimitotic Agents Phase 4,Phase 2,Phase 1
39 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Phase 1
40 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 1
41 Antineoplastic Agents, Phytogenic Phase 4,Phase 2,Phase 1
42 Folate Nutraceutical Phase 4,Phase 2
43 Vitamin B9 Nutraceutical Phase 4,Phase 2
44
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
45
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
46
Melphalan Approved Phase 2 148-82-3 4053 460612
47
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
48
Carmustine Approved, Investigational Phase 2 154-93-8 2578
49
Bortezomib Approved, Investigational Phase 2,Phase 1 179324-69-7 387447 93860
50
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837

Interventional clinical trials:

(show all 20)
# Name Status NCT ID Phase Drugs
1 CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL Unknown status NCT02533700 Phase 4 CEOP/IVE/GDP chemotherapy regimen;CEOP chemotherapy regimen for 6 cycles
2 CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for Newly Diagnosed Young Patients With T Cell Lymphoma Active, not recruiting NCT01746992 Phase 4 Cyclophosphamide 750mg/m2;Vincristine 1.4mg/m2;Doxorubicin 50mg/m2;prednisone 60mg/m2;ifosfamide 2000mg/m2;pirarubicin 50mg/m2;pirarubicin 25mg/m2;Etoposide phosphate 100mg/m2;methotrexate 1500mg/m2
3 High-Dose Chemotherapy Given Together With Peripheral Blood Stem Cell Transplant in Treating Patients With Intestinal T-Cell Lymphoma Unknown status NCT00669812 Phase 2 carmustine;cyclophosphamide;cytarabine;doxorubicin hydrochloride;epirubicin hydrochloride;etoposide;ifosfamide;melphalan;methotrexate;prednisolone;vincristine sulfate
4 Study of Bortezomib and Panobinostat in Treating Patients With Relapsed/Refractory Peripheral T-cell Lymphoma or NK/T-cell Lymphoma Completed NCT00901147 Phase 2 panobinostat and bortezomib
5 SAHA + CHOP in Untreated T-cell Non-Hodgkin's Lymphoma Completed NCT00787527 Phase 1, Phase 2 Zolinza (vorinostat);Cyclophosphamide;Doxorubicin;Vincristine;Prednisone
6 CHP-BV Followed by Consolidation With High-dose Therapy / ASCT as Frontline Treatment of Patients With EATL Type 1. Recruiting NCT03217643 Phase 2 Brentuximab Vedotin
7 Combination Chemotherapy and Lenalidomide in Treating Patients With Newly Diagnosed Stage II-IV Peripheral T-cell Non-Hodgkin's Lymphoma Recruiting NCT02561273 Phase 1, Phase 2 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Lenalidomide;Prednisone;Vincristine Sulfate
8 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
9 Brentuximab Vedotin and Combination Chemotherapy in Treating Patients With CD30-Positive Peripheral T-cell Lymphoma Recruiting NCT03113500 Phase 2 Brentuximab Vedotin;Cyclophosphamide;Doxorubicin;Etoposide;Prednisone
10 A Phase II Study of Single Agent Brentuximab Vedotin in Relapsed/Refractory CD30 Low (<10%) Mature T Cell Lymphoma (TCL) Recruiting NCT02588651 Phase 2 Brentuximab vedotin
11 CAR-pNK Cell Immunotherapy in CD7 Positive Leukemia and Lymphoma Recruiting NCT02742727 Phase 1, Phase 2
12 CHOP vs GEM-P in 1st Line Treatment of T-cell Lymphoma, Multicentre Phase II Study Active, not recruiting NCT01719835 Phase 2 Cyclophosphamide;Gemcitabine;Doxorubicin;Vincristine;Prednisolone;methylprednisolone;Cisplatin
13 Combination of Vorinostat and Bortezomib in Relapsed or Refractory T-Cell Non-Hodgkin's Lymphoma Terminated NCT00810576 Phase 2 Vorinostat;Bortezomib
14 Study of MLN8237 in Participants With Advanced Hematological Malignancies Completed NCT00697346 Phase 1 Alisertib
15 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
16 Everolimus and Bortezomib in Treating Patients With Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1 Bortezomib;Everolimus
17 Comparison Between Axial- and Lateral-viewing Capsule Endoscopy in Celiac Disease Recruiting NCT03095573 Not Applicable
18 MAgnetic Resonance Imaging in COeliac Disease Recruiting NCT02551289
19 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715 Not Applicable
20 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409 Not Applicable

Search NIH Clinical Center for Enteropathy-Associated T-Cell Lymphoma

Genetic Tests for Enteropathy-Associated T-Cell Lymphoma

Anatomical Context for Enteropathy-Associated T-Cell Lymphoma

MalaCards organs/tissues related to Enteropathy-Associated T-Cell Lymphoma:

41
T Cells, Small Intestine, Bone, Colon, B Cells, Neutrophil, Lymph Node

Publications for Enteropathy-Associated T-Cell Lymphoma

Articles related to Enteropathy-Associated T-Cell Lymphoma:

(show top 50) (show all 135)
# Title Authors Year
1
Type II enteropathy-associated t-cell lymphoma: A case report and literature review. ( 29888733 )
2018
2
Pathogenesis of Enteropathy-Associated T Cell Lymphoma. ( 29943210 )
2018
3
Coexpression of CD3 and CD20 in Canine Enteropathy-Associated T-cell Lymphoma. ( 29343197 )
2018
4
Intestinal T-cell lymphoma with enteropathy-associated T-cell lymphoma-like features arising in the setting of adult autoimmune enteropathy. ( 29446107 )
2018
5
Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2. ( 28424246 )
2017
6
Type II Enteropathy-Associated T-cell Lymphoma: A Rare Report from Iran. ( 28316767 )
2017
7
Enteropathy-associated T-cell lymphoma presenting as cutaneous deposits. ( 27766624 )
2017
8
A Case of Type II Enteropathy-Associated T-Cell Lymphoma in a Patient With Ulcerative Colitis. ( 28572650 )
2017
9
Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations. ( 27600764 )
2016
10
High myeloid-derived suppressor cell frequencies in the duodenum are associated with enteropathy associated T-cell lymphoma and its precursor lesions. ( 27341082 )
2016
11
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 27772962 )
2016
12
Type I enteropathy-associated T-cell lymphoma in the colon of a 29-year-old patient and a brief literature review. ( 26955284 )
2016
13
Enteropathy-associated T-cell lymphoma: An extremely rare cause of chronic diarrhoea. ( 27326953 )
2016
14
Eosinophil-Rich Acute Febrile Neutrophilic Dermatosis in a Patient With Enteropathy-Associated T-cell Lymphoma, Type 1. ( 27097333 )
2016
15
Enteropathy-Associated T-Cell Lymphoma. ( 27900603 )
2016
16
Establishment of a Patient-Derived Xenograft of Canine Enteropathy-Associated T-Cell Lymphoma, Large Cell Type. ( 28011041 )
2016
17
Enteropathy-associated T-cell lymphoma in the US: higher incidence and poorer survival among Asians. ( 26104061 )
2015
18
Cross Lineage Rearrangement in Feline Enteropathy-Associated T-cell Lymphoma. ( 26215761 )
2015
19
A new and validated clinical prognostic model (EPI) for Enteropathy Associated T-cell Lymphoma. ( 25779949 )
2015
20
Enteropathy-associated T-cell lymphoma: improving treatment strategies. ( 25925928 )
2015
21
Enteropathy-associated T-cell lymphoma: involvement of the gastrointestinal tract from the duodenum to the rectum. ( 26115398 )
2015
22
Primary Enteropathy-Associated T-Cell Lymphoma Type 2: An Emerging Entity? ( 26068772 )
2015
23
Enteropathy-associated T-cell lymphoma in childhood: a case report and review of the literature. ( 25629982 )
2015
24
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 26226842 )
2015
25
Discordant and metachronous Non-Hodgkin's lymphomas: Enteropathy-associated T-cell lymphoma, Type II with aberrant expression of CD4 presenting several years after a mucosa-associated lymphoid tissue type B-cell lymphoma. ( 26549098 )
2015
26
A rare but potentially fatal cause of diarrhoea and weight loss: enteropathy-associated T-cell lymphoma. ( 25568262 )
2015
27
Endoscopic Findings of Enteropathy-Associated T-Cell Lymphoma Type II: A Case Series. ( 26260757 )
2015
28
Treatment response in Enteropathy Associated T-cell Lymphoma; survival in a large multicenter cohort. ( 25716069 )
2015
29
An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity. ( 25308040 )
2014
30
Enteropathy-associated T-cell lymphoma, type II (monomorphic variant). ( 24822244 )
2014
31
Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses. ( 24943780 )
2014
32
Enteropathy-associated T-cell lymphoma presenting with gastrointestinal tract symptoms: A report of two cases and review of diagnostic challenges and clinicopathological correlation. ( 24959225 )
2014
33
Detailed clinicopathological characteristics and possible lymphomagenesis of type II intestinal enteropathy-associated T-cell lymphoma in Japan. ( 24746558 )
2014
34
Enteropathy-associated T cell lymphoma as a complication of silent celiac disease. ( 25568762 )
2014
35
Enteropathy associated T cell lymphoma - a case report of an uncommon extranodal T cell lymphoma. ( 25478355 )
2014
36
Successful Diagnosis of Type II Enteropathy-associated T-cell Lymphoma Using Flow Cytometry and the Cell Block Technique of Celomic Fluid Manifesting as Massive Pyoid Ascites that Could Not Be Diagnosed via Emergency Laparotomy. ( 24429453 )
2014
37
Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel haemorrhage and perforation. ( 24876396 )
2014
38
Profile of CD103 Expression in T-cell Neoplasms: Immunoreactivity Is Not Restricted to Enteropathy-associated T-cell Lymphoma. ( 25025448 )
2014
39
A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity. ( 25588633 )
2014
40
Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease. ( 26157854 )
2014
41
Classic versus type II enteropathy-associated T-cell lymphoma: diagnostic considerations. ( 23991736 )
2013
42
Hemophagocytic lymphohistiocytosis: an uncommon presentation of enteropathy-associated T-cell lymphoma. ( 23530098 )
2013
43
Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT. ( 23361910 )
2013
44
Challenges in the diagnosis of enteropathy-associated T cell lymphoma. ( 23964351 )
2013
45
Obscure gastrointestinal bleeding and video capsule retention due to enteropathy-associated T-cell lymphoma. ( 24719605 )
2013
46
A case of colon perforation due to enteropathy-associated T-cell lymphoma. ( 23555174 )
2013
47
Clinically undiagnosed enteropathy associated T-cell lymphoma type II presenting with prolonged lower gastrointestinal tract symptoms: report of an autopsy case and review of diagnostic challenges and clinicopathological correlation. ( 23450273 )
2013
48
Brentuximab in the treatment of CD30-positive enteropathy-associated T-cell lymphoma. ( 23411380 )
2013
49
Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. ( 23313469 )
2013
50
Enteropathy-associated T-cell lymphoma diagnosed and followed-up by using double-balloon enteroscopy. ( 23541443 )
2013

Variations for Enteropathy-Associated T-Cell Lymphoma

Expression for Enteropathy-Associated T-Cell Lymphoma

Search GEO for disease gene expression data for Enteropathy-Associated T-Cell Lymphoma.

Pathways for Enteropathy-Associated T-Cell Lymphoma

Pathways related to Enteropathy-Associated T-Cell Lymphoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.28 CCL11 CD3G ITGAE
2 10.3 CD3G ITGAE

GO Terms for Enteropathy-Associated T-Cell Lymphoma

Sources for Enteropathy-Associated T-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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