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EATL
MCID: ENT008
MIFTS: 36

Enteropathy-Associated T-Cell Lymphoma (EATL)

Categories: Blood diseases, Cancer diseases, Gastrointestinal diseases, Immune diseases, Rare 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 20 58 71
High-Grade Pleomorphic Peripheral T-Cell Lymphoma 20
Enteropathy-Type T-Cell Lymphoma 58
Intestinal T-Cell Lymphoma 58
Eatcl 20
Eatl 58
Ettl 58

Characteristics:

Orphanet epidemiological data:

58
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: 32 33
Orphanet: 58  
Rare gastroenterological diseases
Rare haematological diseases


External Ids:

ICD10 32 C86.2
MESH via Orphanet 45 D058527
ICD10 via Orphanet 33 C86.2
UMLS via Orphanet 72 C0456889
Orphanet 58 ORPHA86880
UMLS 71 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 celiac disease 1 and lymphoma. An important gene associated with Enteropathy-Associated T-Cell Lymphoma is SETD2 (SET Domain Containing 2, Histone Lysine Methyltransferase), and among its related pathways/superpathways are Akt Signaling and Reelin Pathway (Cajal-Retzius cells). The drugs Methotrexate and Pirarubicin have been mentioned in the context of this disorder. Affiliated tissues include t cells, small intestine and colon, and related phenotypes are hematopoietic system and immune 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 top 50) (show all 110)
# Related Disease Score Top Affiliating Genes
1 celiac disease 1 11.0
2 lymphoma 10.9
3 refractory celiac disease 10.7
4 peripheral t-cell lymphoma 10.7
5 lymphoma, hodgkin, classic 10.6
6 lymphoma, non-hodgkin, familial 10.6
7 intestinal perforation 10.6
8 diarrhea 10.4
9 intestinal obstruction 10.4
10 b-cell lymphoma 10.3
11 gastrointestinal lymphoma 10.3
12 t-cell lymphoma, subcutaneous panniculitis-like 10.2
13 adult t-cell leukemia 10.2
14 inflammatory bowel disease 10.2
15 anaplastic large cell lymphoma 10.2
16 toxic shock syndrome 10.2
17 acute kidney failure 10.2
18 fibrosarcoma of bone 10.2
19 histiocytosis 10.2
20 t-cell lymphoblastic leukemia/lymphoma 10.2
21 peptic ulcer disease 10.2
22 reticulosarcoma 10.2
23 ulcerative colitis 10.2
24 hypereosinophilic syndrome 10.2
25 autoimmune enteropathy 10.2
26 leukemia, t-cell, chronic 10.2
27 t-cell non-hodgkin lymphoma 10.2
28 severe immune-mediated enteropathy 10.2
29 peritonitis 10.2
30 marginal zone b-cell lymphoma 10.1
31 nodal marginal zone lymphoma 10.1
32 protein-losing enteropathy 10.1
33 hypothyroidism 10.1
34 splenomegaly 10.1
35 b-cell non-hodgkin lymphoma 10.1
36 atrial standstill 1 10.1
37 autoimmune disease 10.1
38 burkitt lymphoma 10.1
39 intussusception 10.1
40 anemia, autoimmune hemolytic 10.1
41 hypereosinophilic syndrome, idiopathic 10.1
42 neutrophilic dermatosis, acute febrile 10.1
43 myocardial infarction 10.1
44 cholangitis, primary sclerosing 10.1
45 deficiency anemia 10.1
46 hemophagocytic lymphohistiocytosis 10.1
47 mature t-cell and nk-cell lymphoma 10.1
48 diffuse large b-cell lymphoma 10.1
49 colitis 10.1
50 collagenous colitis 10.1

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:

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
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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 88)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
2
Pirarubicin Investigational Phase 4 72496-41-4
3
Melphalan Approved Phase 2 148-82-3 4053 460612
4
Epirubicin Approved Phase 2 56420-45-2 41867
5
Cytarabine Approved, Investigational Phase 2 147-94-4 6253
6
Panobinostat Approved, Investigational Phase 2 404950-80-7 6918837
7
Bortezomib Approved, Investigational Phase 2 179324-69-7 387447 93860
8
Carmustine Approved, Investigational Phase 2 154-93-8 2578
9
Levoleucovorin Approved, Investigational Phase 1, Phase 2 68538-85-2 149436
10
Pembrolizumab Approved Phase 1, Phase 2 1374853-91-4
11
Daunorubicin Approved Phase 2 20830-81-3 30323
12
Vincristine Approved, Investigational Phase 2 2068-78-2, 57-22-7 5978
13
Prednisolone Approved, Vet_approved Phase 2 50-24-8 5755
14
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
15
Etoposide Approved Phase 2 33419-42-0 36462
16
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
17 Prednisolone acetate Approved, Vet_approved Phase 2 52-21-1
18
Ifosfamide Approved Phase 2 3778-73-2 3690
19
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
20
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
21
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0
22
Prednisone Approved, Vet_approved Phase 2 53-03-2 5865
23
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
24
Gemcitabine Approved Phase 2 95058-81-4 60750
25
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
26
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
27
Lenalidomide Approved Phase 1, Phase 2 191732-72-6 216326
28
nivolumab Approved Phase 2 946414-94-4
29
Folic acid Approved, Nutraceutical, Vet_approved Phase 1, Phase 2 59-30-3 6037
30
Prednisolone hemisuccinate Experimental Phase 2 2920-86-7
31
Cortisone Experimental Phase 1, Phase 2 53-06-5 222786
32 Histone Deacetylase Inhibitors Phase 2
33 Proteasome Inhibitors Phase 2
34 Folic Acid Antagonists Phase 1, Phase 2
35 Vitamin B Complex Phase 1, Phase 2
36 Vitamin B9 Phase 1, Phase 2
37 Folate Phase 1, Phase 2
38 10-deazaaminopterin Phase 1, Phase 2
39 Immunoconjugates Phase 2
40 Immunoglobulins Phase 2
41 Antibodies Phase 2
42 Antibodies, Monoclonal Phase 2
43 Immunoglobulins, Intravenous Phase 2
44 Immunosuppressive Agents Phase 1, Phase 2
45 Antibiotics, Antitubercular Phase 1, Phase 2
46 Hormone Antagonists Phase 1, Phase 2
47 Methylprednisolone Acetate Phase 2
48 Immunologic Factors Phase 1, Phase 2
49 Anti-Inflammatory Agents Phase 1, Phase 2
50 Hormones Phase 1, Phase 2

Interventional clinical trials:

(show all 19)
# Name Status NCT ID Phase Drugs
1 An Open-label,Multicenter Randomised Study of CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for the New Diagnosed Young Patients With T Cell Non-hodgkin Lymphoma Unknown status 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
2 A Phase II Evaluation of High Dose Chemotherapy and Autologous Stem Cell Transplantation for Intestinal T-cell Lymphomas Unknown status NCT00669812 Phase 2 carmustine;cyclophosphamide;cytarabine;doxorubicin hydrochloride;epirubicin hydrochloride;etoposide;ifosfamide;melphalan;methotrexate;prednisolone;vincristine sulfate
3 Chimeric Antigen Receptor-Modified pNK Cells for CD7 Positive Relapsed or Refractory Leukemia and Lymphoma Unknown status NCT02742727 Phase 1, Phase 2
4 An Open-label Phase 2 Study of Intravenous Bortezomib and Oral Panobinostat (LBH589) in Adult Patients With Relapsed/Refractory Peripheral T-cell Lymphoma or NK/T-cell Lymphoma After Failure of Conventional Chemotherapy Completed NCT00901147 Phase 2 panobinostat and bortezomib
5 Phase 2 Study of Brentuximab Vedotin Associated With CHP Followed by Consolidation With High-dose Therapy / Autologous Stem-cell Transplantation as Frontline Treatment of Patients With Enteropathy-associated T-cell Lymphoma Type 1. Recruiting NCT03217643 Phase 2 Brentuximab Vedotin
6 A Phase 1/2 Study of Pembrolizumab Plus Pralatrexate for Treatment of Relapsed or Refractory Peripheral T-Cell Lymphomas Recruiting NCT03598998 Phase 1, Phase 2 Pralatrexate
7 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
8 A Phase 2 Study of Brentuximab Vedotin Plus Cyclophosphamide, Doxorubicin, Etoposide, and Prednisone (CHEP-BV) Followed by BV Consolidation in Patients With CD30-Positive Peripheral T-Cell Lymphomas Recruiting NCT03113500 Phase 2 Brentuximab Vedotin;Cyclophosphamide;Doxorubicin;Doxorubicin Hydrochloride;Etoposide;Etoposide Phosphate;Prednisone
9 CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With Peripheral T-cell Lymphoma (PTCL) Active, not recruiting NCT02533700 Phase 2 CEOP/IVE/GDP chemotherapy regimen;CEOP chemotherapy regimen for 6 cycles
10 A Phase I/II Trial of CHOEP Chemotherapy Plus Lenalidomide as Front Line Therapy for Patients With Stage II, III and IV Peripheral T-Cell Non-Hodgkin's Lymphoma Active, not recruiting NCT02561273 Phase 1, Phase 2 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Lenalidomide;Prednisone;Vincristine Sulfate
11 CHEMO-T: Cyclophosphamide, Doxorubicin, Vincristine and Prednisolone (CHOP) Versus Gemcitabine, Cisplatin and Methyl Prednisolone (GEM-P) in the First Line Treatment Of T-cell Lymphoma,a Multicentre Randomised Phase II Study Active, not recruiting NCT01719835 Phase 2 Cyclophosphamide;Gemcitabine;Doxorubicin;Vincristine;Prednisolone;methylprednisolone;Cisplatin
12 Phase 2 Single-Arm, Open-Label Study of Nivolumab in Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma (PTCL) Terminated NCT03075553 Phase 2
13 An Open-label, Phase 1 Study of MLN8237, a Novel Aurora A Kinase Inhibitor, in Patients With Advanced Hematological Malignancies Completed NCT00697346 Phase 1 Alisertib
14 Phase I Study of Romidepsin (ISTODAX®) Plus ICE for Patients With Relapsed or Refractory Peripheral T-Cell Lymphoma Completed NCT01590732 Phase 1 Carboplatin;Etoposide;Ifosfamide;Romidepsin
15 Anti-CD30 CAR T Cells With Fully-human Binding Domains for Treating CD30-expressing Lymphomas Including Anaplastic Large Cell Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
16 Early Clinical Study on Increasing Dose and Open Label of T Cell Injection Targeting CD7 Autologous Chimeric Antigen Receptor in the Treatment of Relapsed / Refractory CD7 Positive Hemolymph System Malignancies Recruiting NCT04480788 Phase 1
17 A Phase 1 Study of Doxorubicin, CC-486 (5-azacitidine), Romidepsin, and Duvelisib (hARD) for T-cell Lymphoma Not yet recruiting NCT04639843 Phase 1 CC-486 (5-azacitidine);Duvelisib;Romidepsin;Doxorubicin
18 A Pilot Feasibility Trial of the Tolerability of Oral Salvia Hispanica and Its Effect on Blood Fatty Acids and Stool Microbiome in Patients With Treated Non-Hodgkin Lymphoma Completed NCT02652715
19 Effect of Vitamin D Replacement on Tumor Response and Survival Parameters for Vitamin D Insufficient Patients With Cancer 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:

40
T Cells, Small Intestine, Colon, Lymph Node, Myeloid, Bone Marrow, Appendix
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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 521)
# Title Authors PMID Year
1
Immunophenotypic Spectrum and Genomic Landscape of Refractory Celiac Disease Type II. 61
33544565 2021
2
Intestinal T-cell lymphoma presenting as colonic perforation in the setting of ulcerative colitis: a case report. 61
33174156 2021
3
18F-FDG PET/CT findings in a rare monomorphic epitheliotropic intestinal T cell lymphoma. 61
32632459 2021
4
Oncogenetic landscape of lymphomagenesis in coeliac disease. 61
33579790 2021
5
Lymphomatous effusion of monomorphic epitheliotropic intestinal T-cell lymphoma is characterized by azurophilic granules and is a dismal sign: Report of two new cases with literature review. 61
33387400 2021
6
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma (MEITL) Within a Meckel's Diverticulum. 61
32830541 2021
7
Multiple cerebral lesions in a patient with refractory celiac disease: A case report. 61
33384556 2020
8
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Secondary Cutaneous Involvement: A Diagnostic Challenge. 61
33264131 2020
9
Organizing pneumonia associated with T-cell lymphoma. 61
33133608 2020
10
Long-term remission in multiply relapsed enteropathy-associated T-cell lymphoma following CD30 CAR T-cell therapy. 61
33259598 2020
11
Monitoring of tumor cells by flow cytometry permits rapid evaluation of disease progression in monomorphic epitheliotropic intestinal T-cell lymphoma. 61
33259698 2020
12
Monomorphic epitheliotropic intestinal T-cell lymphoma in a patient with chronic diarrhea and steatorrhea. 61
33277115 2020
13
Tumour-specific microRNA expression pattern in canine intestinal T-cell-lymphomas. 61
31997569 2020
14
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma in Asia Frequently Shows SETD2 Alterations. 61
33260897 2020
15
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature. 61
33334797 2020
16
Early colon cancer with enteropathy-associated T-cell lymphoma involving the whole gastrointestinal tract: A case report. 61
33344574 2020
17
Effectiveness of capsule endoscopy and double-balloon enteroscopy in suspected complicated celiac disease. 61
33189853 2020
18
[CD20-positive T cell lymphoma: clinicopathological features of five cases]. 61
32992416 2020
19
Endoscopic and clinicopathological characteristics of colorectal T/NK cell lymphoma. 61
33087157 2020
20
Whole-genome sequencing reveals potent therapeutic strategy for monomorphic epitheliotropic intestinal T-cell lymphoma. 61
33017466 2020
21
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Invades Brain. 61
33028779 2020
22
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma Involving the Lung and Brain: A Rare Case Study. 61
32641648 2020
23
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma of the Stomach: Two Case Reports and a Literature Review. 61
32856508 2020
24
Intestinal T-cell lymphoma in a coati (Nasua nasua) - Short communication. 61
32857708 2020
25
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Extraintestinal Areas of Peripheral T-Cell Lymphoma Involvement. 61
32983716 2020
26
Incidental Diagnosis of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Case Report. 61
33005508 2020
27
Preliminary evidence of imaging of chemokine receptor-4-targeted PET/CT with [68Ga]pentixafor in non-Hodgkin lymphoma: comparison to [18F]FDG. 61
32757068 2020
28
Refractory celiac disease type II: An atypical case highlighting limitations of the current classification system. 61
32010998 2020
29
Recent Advances in Digestive Tract Tumors: Updates From the 5th Edition of the World Health Organization "Blue Book". 61
32886739 2020
30
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Rare Case of Diffuse Ulcers in the Intestine. 61
32668339 2020
31
Monomorphic epitheliotropic intestinal T-cell lymphoma of the duodenum: an aggressive disease. 61
32536535 2020
32
Type II enteropathy-associated T cell lymphoma in the duodenum: A rare case report. 61
32501967 2020
33
Monomorphic Epitheliotropic Intestinal T Cell Lymphoma of the Appendix: a Case Report and Review of Literature. 61
31970655 2020
34
Epidemiological, pathological and immunohistochemical aspects of 125 cases of feline lymphoma in Southern Brazil. 61
31461200 2020
35
Deregulation of miRNAs-cMYC circuits is a key event in refractory celiac disease type-2 lymphomagenesis. 61
32420596 2020
36
Update on lymphoproliferative disorders of the gastrointestinal tract: disease spectrum from indolent lymphoproliferations to aggressive lymphomas. 61
31773249 2020
37
Equine Intestinal Lymphoma: Clinical-Pathological Features, Immunophenotype, and Survival. 61
32202217 2020
38
Chidamide based combination regimen for treatment of monomorphic epitheliotropic intestinal T cell lymphoma following radical operation: Two case reports. 61
32337203 2020
39
Incidence and outcomes of rare T cell lymphomas from the T Cell Project: hepatosplenic, enteropathy associated and peripheral gamma delta T cell lymphomas. 61
31709579 2020
40
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders. 61
31522873 2020
41
[Monomorphic epitheliotropic intestinal T-cell lymphoma: a clinicopathological analysis of twelve cases]. 61
31914529 2020
42
Enteropathy-associated T-cell Lymphoma (EATL) with intracranial metastasis : a rare and dismal condition. 61
32233276 2020
43
Intestinal T-cell lymphoma, NOS, presenting with sole peritoneal and mucosal lymphomatosis throughout abdominal cavity. 61
32981915 2020
44
The Intracellular Intensity of CD3 on Aberrant Intraepithelial Lymphocytes Is a Prognostic Factor of the Progression to Overt Lymphoma in Refractory Celiac Disease Type II (Pre-Enteropathy-Associated T Cell Lymphoma). 61
32015237 2020
45
Celiac disease in adults. 61
32942892 2020
46
T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update. 61
31727264 2020
47
Characterization of the fecal microbiome in cats with inflammatory bowel disease or alimentary small cell lymphoma. 61
31844119 2019
48
Monomorphic Epitheliotropic Intestinal T cell Lymphoma: a Rare Cause of Chronic Diarrhea. 61
30761475 2019
49
Anaplastic Large T-Cell Lymphoma in the Intestine of Dogs. 61
31170900 2019
50
Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma. 61
31493002 2019
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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 63
Show member pathways
 12.28 ITGAE CD3G CCL11
2
Reelin Pathway (Cajal-Retzius cells) 63
10.63 ITGAE CD3G
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GO Terms for Enteropathy-Associated T-Cell Lymphoma

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

# Name GO ID Score Top Affiliating Genes
1 external side of plasma membrane GO:0009897 8.62 ITGAE CD3G
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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
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 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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