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EATL
MCID: ENT008
MIFTS: 35

Enteropathy-Associated T-Cell Lymphoma (EATL) malady

Categories: Rare diseases, Cancer diseases, Gastrointestinal diseases, Blood diseases, Immune diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Enteropathy-Associated T-Cell Lymphoma

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Aliases & Descriptions for Enteropathy-Associated T-Cell Lymphoma:

Name: Enteropathy-Associated T-Cell Lymphoma 50 56 69
High-Grade Pleomorphic Peripheral T-Cell Lymphoma 50
Enteropathy-Type T-Cell Lymphoma 56
Intestinal T-Cell Lymphoma 56
Eatcl 50
Eatl 56
Ettl 56

Characteristics:

Orphanet epidemiological data:

56
enteropathy-associated t-cell lymphoma
Age of onset: Adult;

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Gastrointestinal diseases Blood diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 34 33
Orphanet: 56  
Rare gastroenterological diseases
Rare haematological diseases


External Ids:

Orphanet 56 ORPHA86880
MESH via Orphanet 43 D058527
UMLS via Orphanet 70 C0456889
ICD10 via Orphanet 34 C86.2
ICD10 33 C86.2
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Summaries for Enteropathy-Associated T-Cell Lymphoma

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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. 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 ERK Signaling and Akt Signaling. The drugs Cyclophosphamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include t cells, colon and b cells.

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Related Diseases for Enteropathy-Associated T-Cell Lymphoma

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

(show all 22)
id Related Disease Score Top Affiliating Genes
1 lymphoma 10.9
2 celiac disease 10.5
3 refractory celiac disease 10.3
4 malignant histiocytosis 10.0
5 b-cell lymphomas 10.0
6 mucositis 10.0
7 histiocytosis 10.0
8 apricot allergy 9.9 CCL11 ITGAE
9 myocardial infarction 9.9
10 cholangitis 9.9
11 autoimmune enteropathy 9.9
12 cardiomyopathy 9.9
13 hemolytic anemia 9.9
14 hemophagocytic lymphohistiocytosis 9.9
15 peripheral t-cell lymphoma 9.9
16 jejunoileitis 9.9
17 obstructive jaundice 9.9
18 sclerosing cholangitis 9.9
19 intestinal perforation 9.9
20 restrictive cardiomyopathy 9.9
21 autoimmune hemolytic anemia 9.9
22 eosinophilic granulomatosis with polyangiitis 9.5 CCL11 CD3G ITGAE

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



Diseases related to Enteropathy-Associated T-Cell Lymphoma
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Symptoms & Phenotypes for Enteropathy-Associated T-Cell Lymphoma

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Drugs & Therapeutics for Enteropathy-Associated T-Cell Lymphoma

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

(show top 50) (show all 94)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Cyclophosphamide Approved, Investigational Phase 4,Phase 2,Phase 1 50-18-0, 6055-19-2 2907
2
Doxorubicin Approved, Investigational Phase 4,Phase 2,Phase 1 23214-92-8 31703
3
Etoposide Approved Phase 4,Phase 2,Phase 1 33419-42-0 36462
4
Ifosfamide Approved Phase 4,Phase 2,Phase 1 3778-73-2 3690
5
Methotrexate Approved Phase 4,Phase 2 1959-05-2, 59-05-2 126941
6
Vincristine Approved, Investigational Phase 4,Phase 2,Phase 1 2068-78-2, 57-22-7 5978
7
Epirubicin Approved Phase 4,Phase 2 56420-45-2 41867
8
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2 53-03-2 5865
9
Mechlorethamine Approved Phase 4,Phase 1 51-75-2 4033
10
Cisplatin Approved Phase 4 15663-27-1 84093 441203 2767
11
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
12
Gemcitabine Approved Phase 4 95058-81-4 60750
13
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2 59-30-3 6037
14
leucovorin Approved, Nutraceutical Phase 4,Phase 2 58-05-9 54575, 6560146 143
15
Doxil Approved June 1999 Phase 4,Phase 2,Phase 1 31703
16
Pirarubicin Investigational Phase 4 72496-41-4
17 Alkylating Agents Phase 4,Phase 2,Phase 1
18 Anti-Bacterial Agents Phase 4,Phase 2,Phase 1
19 Antibiotics, Antitubercular Phase 4,Phase 2,Phase 1
20 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 1
21 Antimetabolites Phase 4,Phase 2
22 Antimetabolites, Antineoplastic Phase 4,Phase 2
23 Antimitotic Agents Phase 4,Phase 2,Phase 1
24 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Phase 1
25 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 1
26 Antineoplastic Agents, Phytogenic Phase 4,Phase 2,Phase 1
27 Antirheumatic Agents Phase 4,Phase 2,Phase 1
28 Dermatologic Agents Phase 4,Phase 2
29 Etoposide phosphate Phase 4,Phase 2,Phase 1
30 Folic Acid Antagonists Phase 4,Phase 2
31 glucocorticoids Phase 4,Phase 2,Phase 1
32 Hormone Antagonists Phase 4,Phase 2,Phase 1
33 Hormones Phase 4,Phase 2,Phase 1
34 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 1
35 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
36 Isophosphamide mustard Phase 4,Phase 2,Phase 1
37 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
38 Topoisomerase Inhibitors Phase 4,Phase 2,Phase 1
39 Vitamin B Complex Phase 4,Phase 2
40 BB 1101 Phase 4
41 Dexamethasone 21-phosphate Phase 4
42 Dexamethasone acetate Phase 4 1177-87-3
43 Folate Nutraceutical Phase 4,Phase 2
44 Vitamin B9 Nutraceutical Phase 4,Phase 2
45
Carmustine Approved Phase 2 154-93-8 2578
46
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
47
Melphalan Approved Phase 2 148-82-3 4053 460612
48
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
49
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
50
Bortezomib Approved, Investigational Phase 2,Phase 1 179324-69-7 387447 93860

Interventional clinical trials:

(show all 19)
id Name Status NCT ID Phase
1 CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for Newly Diagnosed Young Patients With T Cell Lymphoma Recruiting NCT01746992 Phase 4
2 CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL Recruiting NCT02533700 Phase 4
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
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
5 SAHA + CHOP in Untreated T-cell Non-Hodgkin's Lymphoma Completed NCT00787527 Phase 1, Phase 2
6 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
7 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
8 A Phase II Study of Single Agent Brentuximab Vedotin in Relapsed/Refractory CD30 Low (<10%) Mature T Cell Lymphoma (TCL) Recruiting NCT02588651 Phase 2
9 Brentuximab Vedotin and Combination Chemotherapy in Treating Patients With CD30-Positive Peripheral T-cell Lymphoma Recruiting NCT03113500 Phase 2
10 CAR-pNK Cell Immunotherapy in CD7 Positive Leukemia and Lymphoma Recruiting NCT02742727 Phase 1, Phase 2
11 Combination of Vorinostat and Bortezomib in Relapsed or Refractory T-Cell Non-Hodgkin's Lymphoma Terminated NCT00810576 Phase 2
12 Study of MLN8237 in Participants With Advanced Hematological Malignancies Completed NCT00697346 Phase 1
13 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1
14 Phase I Study of Romidepsin Plus ICE for Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma Active, not recruiting NCT01590732 Phase 1
15 Everolimus and Bortezomib in Treating Patients With Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1
16 Comparison Between Axial- and Lateral-viewing Capsule Endoscopy in Celiac Disease Recruiting NCT03095573
17 MAgnetic Resonance Imaging in COeliac Disease Recruiting NCT02551289
18 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Recruiting NCT02652715
19 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409

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

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Genetic Tests for Enteropathy-Associated T-Cell Lymphoma

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Anatomical Context for Enteropathy-Associated T-Cell Lymphoma

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

39
T Cells, Colon, B Cells, Neutrophil, Nk Cells, Lymph Node, Brain
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Publications for Enteropathy-Associated T-Cell Lymphoma

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Articles related to Enteropathy-Associated T-Cell Lymphoma:

(show top 50) (show all 128)
id Title Authors Year
1
Enteropathy-associated T-cell lymphoma presenting as cutaneous deposits. ( 27766624 )
2017
2
Eosinophil-Rich Acute Febrile Neutrophilic Dermatosis in a Patient With Enteropathy-Associated T-cell Lymphoma, Type 1. ( 27097333 )
2016
3
Establishment of a Patient-Derived Xenograft of Canine Enteropathy-Associated T-Cell Lymphoma, Large Cell Type. ( 28011041 )
2016
4
High myeloid-derived suppressor cell frequencies in the duodenum are associated with enteropathy associated T-cell lymphoma and its precursor lesions. ( 27341082 )
2016
5
Type I enteropathy-associated T-cell lymphoma in the colon of a 29-year-old patient and a brief literature review. ( 26955284 )
2016
6
Enteropathy-associated T-cell lymphoma: An extremely rare cause of chronic diarrhoea. ( 27326953 )
2016
7
Enteropathy-Associated T-Cell Lymphoma. ( 27900603 )
2016
8
Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations. ( 27600764 )
2016
9
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 27772962 )
2016
10
A rare but potentially fatal cause of diarrhoea and weight loss: enteropathy-associated T-cell lymphoma. ( 25568262 )
2015
11
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
12
Cross Lineage Rearrangement in Feline Enteropathy-Associated T-cell Lymphoma. ( 26215761 )
2015
13
Primary Enteropathy-Associated T-Cell Lymphoma Type 2: An Emerging Entity? ( 26068772 )
2015
14
Treatment response in Enteropathy Associated T-cell Lymphoma; survival in a large multicenter cohort. ( 25716069 )
2015
15
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 26226842 )
2015
16
A new and validated clinical prognostic model (EPI) for Enteropathy Associated T-cell Lymphoma. ( 25779949 )
2015
17
Enteropathy-associated T-cell lymphoma: involvement of the gastrointestinal tract from the duodenum to the rectum. ( 26115398 )
2015
18
Enteropathy-associated T-cell lymphoma: improving treatment strategies. ( 25925928 )
2015
19
Enteropathy-associated T-cell lymphoma in the US: higher incidence and poorer survival among Asians. ( 26104061 )
2015
20
Endoscopic Findings of Enteropathy-Associated T-Cell Lymphoma Type II: A Case Series. ( 26260757 )
2015
21
Enteropathy-associated T-cell lymphoma in childhood: a case report and review of the literature. ( 25629982 )
2015
22
Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease. ( 26157854 )
2014
23
Enteropathy associated T cell lymphoma - a case report of an uncommon extranodal T cell lymphoma. ( 25478355 )
2014
24
Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel haemorrhage and perforation. ( 24876396 )
2014
25
Profile of CD103 Expression in T-cell Neoplasms: Immunoreactivity Is Not Restricted to Enteropathy-associated T-cell Lymphoma. ( 25025448 )
2014
26
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
27
A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity. ( 25588633 )
2014
28
Enteropathy-associated T cell lymphoma as a complication of silent celiac disease. ( 25568762 )
2014
29
An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity. ( 25308040 )
2014
30
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
31
Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses. ( 24943780 )
2014
32
Enteropathy-associated T-cell lymphoma, type II (monomorphic variant). ( 24822244 )
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 diagnosed and followed-up by using double-balloon enteroscopy. ( 23541443 )
2013
35
Brentuximab in the treatment of CD30-positive enteropathy-associated T-cell lymphoma. ( 23411380 )
2013
36
Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. ( 23313469 )
2013
37
A case of colon perforation due to enteropathy-associated T-cell lymphoma. ( 23555174 )
2013
38
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
39
Celiac disease, enteropathy-associated T-cell lymphoma, and primary sclerosing cholangitis in one patient: a very rare association and review of the literature. ( 24381773 )
2013
40
Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT. ( 23361910 )
2013
41
Obscure gastrointestinal bleeding and video capsule retention due to enteropathy-associated T-cell lymphoma. ( 24719605 )
2013
42
Hemophagocytic lymphohistiocytosis: an uncommon presentation of enteropathy-associated T-cell lymphoma. ( 23530098 )
2013
43
Challenges in the diagnosis of enteropathy-associated T cell lymphoma. ( 23964351 )
2013
44
Classic versus type II enteropathy-associated T-cell lymphoma: diagnostic considerations. ( 23991736 )
2013
45
An unusual enteropathy-associated T-cell lymphoma with MYC translocation arising in a Japanese patient: a case report. ( 22654438 )
2012
46
A case of enteropathy-associated T-cell lymphoma (type I) arising in stomach without refractory celiac disease. ( 23217032 )
2012
47
Supratentorial metastatic enteropathy-associated T-cell lymphoma: A case report and literature review. ( 23230525 )
2012
48
Enteropathy-associated T-cell lymphoma, lacking MHC class II, with immune-privileged site recurrence, presenting as bilateral ocular vitreous humour involvement - a case report. ( 22963578 )
2012
49
Second line chemotherapy in patients with enteropathy-associated T cell lymphoma: a retrospective single center analysis. ( 21520002 )
2012
50
High frequency of fatal haemophagocytic lymphohistiocytosis syndrome in enteropathy-associated T cell lymphoma. ( 22100722 )
2012
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Variations for Enteropathy-Associated T-Cell Lymphoma

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Expression for Enteropathy-Associated T-Cell Lymphoma

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

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

id Super pathways Score Top Affiliating Genes
1
ERK Signaling 60
Show member pathways
 12.97 CCL11 CD3G ITGAE
2
Akt Signaling 60
Show member pathways
 12.28 CCL11 CD3G ITGAE
3
CCR5 Pathway in Macrophages 60
Show member pathways
 12.01 CCL11 CD3G
4
Allograft rejection 1 37
Show member pathways
 11.9 CCL11 CD3G
5
Reelin Pathway (Cajal-Retzius cells) 60
10.63 CD3G ITGAE
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GO Terms for Enteropathy-Associated T-Cell Lymphoma

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

id Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 8.62 CCL11 ITGAE
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Sources for Enteropathy-Associated T-Cell Lymphoma

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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