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MCID: ENT008
MIFTS: 37

Enteropathy-Associated T-Cell Lymphoma

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

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

Name: Enteropathy-Associated T-Cell Lymphoma 49 55 69
High-Grade Pleomorphic Peripheral T-Cell Lymphoma 49
Enteropathy-Type T-Cell Lymphoma 55
Intestinal T-Cell Lymphoma 55
Eatcl 49
Eatl 55
Ettl 55

Characteristics:

Orphanet epidemiological data:

55
enteropathy-associated t-cell lymphoma
Age of onset: Adult;

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Immune diseases Gastrointestinal diseases Blood diseases
See all MalaCards categories (disease lists)
ICD10: 33 32
Orphanet: 55  
Rare gastroenterological diseases
Rare haematological diseases


External Ids:

Orphanet 55 ORPHA86880
MESH via Orphanet 42 D058527
UMLS via Orphanet 70 C0456889
ICD10 via Orphanet 33 C86.2
ICD10 32 C86.2
UMLS 69 C0456889
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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 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 Reelin Pathway (Cajal-Retzius cells). The drugs Doxorubicin and Vincristine have been mentioned in the context of this disorder. Affiliated tissues include t cells, bone and colon, and related phenotype is hematopoietic system.

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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 27)
# Related Disease Score Top Affiliating Genes
1 lymphoma 11.0
2 celiac disease 1 10.5
3 refractory celiac disease 10.3
4 blood group, i system 10.2
5 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
6 b-cell lymphomas 10.1
7 mucositis 10.1
8 histiocytosis 10.1
9 malignant histiocytosis 10.1
10 autoimmune enteropathy 10.1
11 anemia, autoimmune hemolytic 9.9
12 myocardial infarction 9.9
13 cholangitis, primary sclerosing 9.9
14 b-cell expansion with nfkb and t-cell anergy 9.9
15 hemolytic anemia 9.9
16 hemophagocytic lymphohistiocytosis 9.9
17 peripheral t-cell lymphoma 9.9
18 ulcerative colitis 9.9
19 colitis 9.9
20 jejunoileitis 9.9
21 obstructive jaundice 9.9
22 sclerosing cholangitis 9.9
23 intestinal perforation 9.9
24 restrictive cardiomyopathy 9.9
25 cholangitis 9.9
26 autoinflammation, lipodystrophy, and dermatosis syndrome 9.6 CCL11 SETD2
27 gastrointestinal allergy 9.5 CCL11 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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MGI Mouse Phenotypes related to Enteropathy-Associated T-Cell Lymphoma:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 8.92 CCL11 CD3G ITGAE SETD2
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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 87)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 4,Phase 2,Phase 1 23214-92-8 31703
2
Vincristine Approved, Investigational Phase 4,Phase 2,Phase 1 2068-78-2, 57-22-7 5978
3
Etoposide Approved Phase 4,Phase 2,Phase 1 33419-42-0 36462
4
Methotrexate Approved Phase 4,Phase 2 1959-05-2, 59-05-2 126941
5
Cyclophosphamide Approved, Investigational Phase 4,Phase 2,Phase 1 50-18-0, 6055-19-2 2907
6
Ifosfamide Approved Phase 4,Phase 2 3778-73-2 3690
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
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
10
Cisplatin Approved Phase 4 15663-27-1 2767 441203 84093
11
Gemcitabine Approved Phase 4 95058-81-4 60750
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 Isophosphamide mustard Phase 4,Phase 2 0
18 Alkylating Agents Phase 4,Phase 2,Phase 1
19 glucocorticoids Phase 4,Phase 2,Phase 1
20 Folic Acid Antagonists Phase 4,Phase 2
21 Topoisomerase Inhibitors Phase 4,Phase 2,Phase 1
22 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2
23 Hormone Antagonists Phase 4,Phase 2,Phase 1
24 Dermatologic Agents Phase 4,Phase 2,Phase 1
25 Vitamin B Complex 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 Etoposide phosphate Phase 4,Phase 2,Phase 1
30 Immunosuppressive Agents Phase 4,Phase 2,Phase 1
31 Antimetabolites Phase 4,Phase 2
32 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 1
33 Antimetabolites, Antineoplastic Phase 4,Phase 2
34 Antimitotic Agents Phase 4,Phase 2,Phase 1
35 Antibiotics, Antitubercular Phase 4,Phase 2,Phase 1
36 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 1
37 Antineoplastic Agents, Phytogenic Phase 4,Phase 2,Phase 1
38 Antirheumatic Agents Phase 4,Phase 2,Phase 1
39 Dexamethasone acetate Phase 4 1177-87-3
40 BB 1101 Phase 4
41 Folate Nutraceutical Phase 4,Phase 2
42 Vitamin B9 Nutraceutical Phase 4,Phase 2
43
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
44
Melphalan Approved Phase 2 148-82-3 460612 4053
45
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
46
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
47
Carmustine Approved, Investigational Phase 2 154-93-8 2578
48
Bortezomib Approved, Investigational Phase 2,Phase 1 179324-69-7 387447 93860
49
Vorinostat Approved, Investigational Phase 1, Phase 2 149647-78-9 5311
50
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837

Interventional clinical trials:

(show all 19)

# 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 Recruiting 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 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
7 Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma Recruiting NCT03075553 Phase 2
8 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
9 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
10 CAR-pNK Cell Immunotherapy in CD7 Positive Leukemia and Lymphoma Recruiting NCT02742727 Phase 1, Phase 2
11 CHP-BV Followed by Consolidation With High-dose Therapy / ASCT as Frontline Treatment of Patients With EATL Type 1. Not yet recruiting NCT03217643 Phase 2 Brentuximab Vedotin
12 Combination of Vorinostat and Bortezomib in Relapsed or Refractory T-Cell Non-Hodgkin's Lymphoma Terminated NCT00810576 Phase 2 Vorinostat;Bortezomib
13 Study of MLN8237 in Participants With Advanced Hematological Malignancies Completed NCT00697346 Phase 1 Alisertib
14 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
15 Everolimus and Bortezomib in Treating Patients With Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1 Bortezomib;Everolimus
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:

38
T Cells, Bone, Colon, B Cells, Neutrophil, Lymph Node, Nk Cells
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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 133)
# Title Authors Year
1
Intestinal T-cell lymphoma with enteropathy-associated T-cell lymphoma-like features arising in the setting of adult autoimmune enteropathy. ( 29446107 )
2018
2
Coexpression of CD3 and CD20 in Canine Enteropathy-Associated T-cell Lymphoma. ( 29343197 )
2018
3
Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2. ( 28424246 )
2017
4
A Case of Type II Enteropathy-Associated T-Cell Lymphoma in a Patient With Ulcerative Colitis. ( 28572650 )
2017
5
Enteropathy-associated T-cell lymphoma presenting as cutaneous deposits. ( 27766624 )
2017
6
Type II Enteropathy-Associated T-cell Lymphoma: A Rare Report from Iran. ( 28316767 )
2017
7
Type I enteropathy-associated T-cell lymphoma in the colon of a 29-year-old patient and a brief literature review. ( 26955284 )
2016
8
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 27772962 )
2016
9
Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations. ( 27600764 )
2016
10
Enteropathy-Associated T-Cell Lymphoma. ( 27900603 )
2016
11
Eosinophil-Rich Acute Febrile Neutrophilic Dermatosis in a Patient With Enteropathy-Associated T-cell Lymphoma, Type 1. ( 27097333 )
2016
12
Establishment of a Patient-Derived Xenograft of Canine Enteropathy-Associated T-Cell Lymphoma, Large Cell Type. ( 28011041 )
2016
13
Enteropathy-associated T-cell lymphoma: An extremely rare cause of chronic diarrhoea. ( 27326953 )
2016
14
High myeloid-derived suppressor cell frequencies in the duodenum are associated with enteropathy associated T-cell lymphoma and its precursor lesions. ( 27341082 )
2016
15
Primary Enteropathy-Associated T-Cell Lymphoma Type 2: An Emerging Entity? ( 26068772 )
2015
16
A new and validated clinical prognostic model (EPI) for Enteropathy Associated T-cell Lymphoma. ( 25779949 )
2015
17
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
18
Enteropathy-associated T-cell lymphoma: involvement of the gastrointestinal tract from the duodenum to the rectum. ( 26115398 )
2015
19
Enteropathy-associated T-cell lymphoma: improving treatment strategies. ( 25925928 )
2015
20
Enteropathy-associated T-cell lymphoma in childhood: a case report and review of the literature. ( 25629982 )
2015
21
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 26226842 )
2015
22
Endoscopic Findings of Enteropathy-Associated T-Cell Lymphoma Type II: A Case Series. ( 26260757 )
2015
23
Cross Lineage Rearrangement in Feline Enteropathy-Associated T-cell Lymphoma. ( 26215761 )
2015
24
Treatment response in Enteropathy Associated T-cell Lymphoma; survival in a large multicenter cohort. ( 25716069 )
2015
25
Enteropathy-associated T-cell lymphoma in the US: higher incidence and poorer survival among Asians. ( 26104061 )
2015
26
A rare but potentially fatal cause of diarrhoea and weight loss: enteropathy-associated T-cell lymphoma. ( 25568262 )
2015
27
Detailed clinicopathological characteristics and possible lymphomagenesis of type II intestinal enteropathy-associated T-cell lymphoma in Japan. ( 24746558 )
2014
28
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
29
An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity. ( 25308040 )
2014
30
Enteropathy-associated T cell lymphoma as a complication of silent celiac disease. ( 25568762 )
2014
31
Enteropathy associated T cell lymphoma - a case report of an uncommon extranodal T cell lymphoma. ( 25478355 )
2014
32
Enteropathy associated T cell lymphoma-monomorphic variant, presenting as bilateral ovarian masses. ( 24943780 )
2014
33
Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease. ( 26157854 )
2014
34
Profile of CD103 Expression in T-cell Neoplasms: Immunoreactivity Is Not Restricted to Enteropathy-associated T-cell Lymphoma. ( 25025448 )
2014
35
Enteropathy associated T-cell lymphoma presenting with multiple episodes of small bowel haemorrhage and perforation. ( 24876396 )
2014
36
Enteropathy-associated T-cell lymphoma, type II (monomorphic variant). ( 24822244 )
2014
37
A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity. ( 25588633 )
2014
38
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
39
Brentuximab in the treatment of CD30-positive enteropathy-associated T-cell lymphoma. ( 23411380 )
2013
40
Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. ( 23313469 )
2013
41
Enteropathy-associated T-cell lymphoma diagnosed and followed-up by using double-balloon enteroscopy. ( 23541443 )
2013
42
A case of colon perforation due to enteropathy-associated T-cell lymphoma. ( 23555174 )
2013
43
Hemophagocytic lymphohistiocytosis: an uncommon presentation of enteropathy-associated T-cell lymphoma. ( 23530098 )
2013
44
Obscure gastrointestinal bleeding and video capsule retention due to enteropathy-associated T-cell lymphoma. ( 24719605 )
2013
45
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
46
Challenges in the diagnosis of enteropathy-associated T cell lymphoma. ( 23964351 )
2013
47
Classic versus type II enteropathy-associated T-cell lymphoma: diagnostic considerations. ( 23991736 )
2013
48
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
49
Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT. ( 23361910 )
2013
50
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
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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:

# Super pathways Score Top Affiliating Genes
1
Akt Signaling 60
Show member pathways
 12.28 CCL11 CD3G ITGAE
2
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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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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 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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