MCID: ENT008
MIFTS: 39

Enteropathy-Associated T-Cell Lymphoma

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

Aliases & Classifications for Enteropathy-Associated T-Cell Lymphoma

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

Name: Enteropathy-Associated T-Cell Lymphoma 54 60 74
High-Grade Pleomorphic Peripheral T-Cell Lymphoma 54
Enteropathy-Type T-Cell Lymphoma 60
Intestinal T-Cell Lymphoma 60
Eatcl 54
Eatl 60
Ettl 60

Characteristics:

Orphanet epidemiological data:

60

Classifications:



External Ids:

ICD10 34 C86.2
MESH via Orphanet 46 D058527
ICD10 via Orphanet 35 C86.2
UMLS via Orphanet 75 C0456889
Orphanet 60 ORPHA86880
UMLS 74 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 SETD2 (SET Domain Containing 2, Histone Lysine Methyltransferase), and among its related pathways/superpathways are Akt Signaling and G-protein signaling_Regulation of RAC1 activity. The drugs Xalkori and Prednisone have been mentioned in the context of this disorder. Affiliated tissues include t cells, small intestine and testes, and related phenotypes are hematopoietic system and immune system

Wikipedia : 77 Enteropathy-associated T-cell lymphoma (EATL), previously termed enteropathy-associated T-cell lymphoma,... more...

Related Diseases for Enteropathy-Associated T-Cell Lymphoma

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:

47
# 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

FDA approved drugs:

# Drug Name Active Ingredient(s) 19 Company Approval Date
1
Xalkori 19 50 CRIZOTINIB Pfizer August of 2011

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

(show top 50) (show all 108)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2,Early Phase 1 53-03-2 5865
2
Ifosfamide Approved Phase 4,Phase 2,Phase 1,Early Phase 1 3778-73-2 3690
3
Etoposide Approved Phase 4,Phase 2,Phase 1,Early Phase 1 33419-42-0 36462
4
Vincristine Approved, Investigational Phase 4,Phase 2,Phase 1 2068-78-2, 57-22-7 5978
5
Dexamethasone Approved, Investigational, Vet_approved Phase 4,Early Phase 1 50-02-2 5743
6
Epirubicin Approved Phase 4,Phase 2 56420-45-2 41867
7
Cyclophosphamide Approved, Investigational Phase 4,Phase 2,Phase 1,Early Phase 1 50-18-0, 6055-19-2 2907
8
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4,Early Phase 1 1177-87-3
9
Cisplatin Approved Phase 4,Phase 2 15663-27-1 2767 441203 84093
10
Gemcitabine Approved Phase 4,Phase 2 95058-81-4 60750
11
leucovorin Approved Phase 4,Phase 2,Early Phase 1 58-05-9 143 6006
12
Methotrexate Approved Phase 4,Phase 2,Early Phase 1 1959-05-2, 59-05-2 126941
13
Doxorubicin Approved, Investigational Phase 4,Phase 2,Phase 1,Early Phase 1 23214-92-8 31703
14
Mechlorethamine Approved, Investigational Phase 4,Phase 1,Early Phase 1 51-75-2 4033
15
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 2,Early Phase 1 59-30-3 6037
16
Doxil Approved June 1999 Phase 4,Phase 2,Phase 1,Early Phase 1 31703
17
Pirarubicin Investigational Phase 4 72496-41-4
18 Hormones Phase 4,Phase 2,Phase 1,Early Phase 1,Not Applicable
19 Anti-Bacterial Agents Phase 4,Phase 2,Phase 1,Early Phase 1
20 Antimitotic Agents Phase 4,Phase 2,Phase 1
21 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 2,Phase 1,Early Phase 1
22 Alkylating Agents Phase 4,Phase 2,Phase 1,Early Phase 1
23 Topoisomerase Inhibitors Phase 4,Phase 2,Phase 1,Early Phase 1
24 Antineoplastic Agents, Hormonal Phase 4,Phase 2,Phase 1,Early Phase 1
25 Immunosuppressive Agents Phase 4,Phase 2,Phase 1,Early Phase 1
26 Antirheumatic Agents Phase 4,Phase 2,Phase 1,Early Phase 1
27 Anti-Inflammatory Agents Phase 4,Phase 2,Phase 1,Early Phase 1
28 glucocorticoids Phase 4,Phase 2,Phase 1,Early Phase 1
29 Etoposide phosphate Phase 4,Phase 2,Phase 1,Early Phase 1
30 Immunologic Factors Phase 4,Phase 2,Phase 1,Early Phase 1
31 Antibiotics, Antitubercular Phase 4,Phase 2,Phase 1,Early Phase 1
32 BB 1101 Phase 4,Early Phase 1
33 Antineoplastic Agents, Phytogenic Phase 4,Phase 2,Phase 1,Early Phase 1
34 Hormone Antagonists Phase 4,Phase 2,Phase 1,Early Phase 1
35
Isophosphamide mustard Phase 4,Phase 2,Phase 1,Early Phase 1 0
36 Antineoplastic Agents, Alkylating Phase 4,Phase 2,Phase 1,Early Phase 1
37 Vitamin B Complex Phase 4,Phase 2,Early Phase 1
38 Folate Phase 4,Phase 2,Early Phase 1
39 Dermatologic Agents Phase 4,Phase 2,Phase 1,Early Phase 1
40 Vitamin B9 Phase 4,Phase 2,Early Phase 1
41 Antimetabolites, Antineoplastic Phase 4,Phase 2,Phase 1,Early Phase 1
42 Antimetabolites Phase 4,Phase 2,Phase 1,Early Phase 1
43 Nucleic Acid Synthesis Inhibitors Phase 4,Phase 2,Early Phase 1
44 Folic Acid Antagonists Phase 4,Phase 2,Early Phase 1
45
Methylprednisolone Approved, Vet_approved Phase 2 83-43-2 6741
46
Cytarabine Approved, Experimental, Investigational Phase 2 147-94-4, 65-46-3 6253
47
Carmustine Approved, Investigational Phase 2 154-93-8 2578
48
Methylprednisolone hemisuccinate Approved Phase 2 2921-57-5
49
Melphalan Approved Phase 2 148-82-3 460612 4053
50
Prednisolone phosphate Approved, Vet_approved Phase 2 302-25-0

Interventional clinical trials:

(show all 22)
# 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 CAR-pNK Cell Immunotherapy in CD7 Positive Leukemia and Lymphoma Unknown status NCT02742727 Phase 1, Phase 2
5 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
6 SAHA + CHOP in Untreated T-cell Non-Hodgkin's Lymphoma Completed NCT00787527 Phase 1, Phase 2 Zolinza (vorinostat);Cyclophosphamide;Doxorubicin;Vincristine;Prednisone
7 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
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 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
12 Combination Chemotherapy and Lenalidomide in Treating Patients With Newly Diagnosed Stage II-IV Peripheral T-cell Non-Hodgkin's Lymphoma Active, not recruiting NCT02561273 Phase 1, Phase 2 Cyclophosphamide;Doxorubicin Hydrochloride;Etoposide;Lenalidomide;Prednisone;Vincristine Sulfate
13 Combination of Vorinostat and Bortezomib in Relapsed or Refractory T-Cell Non-Hodgkin's Lymphoma Terminated NCT00810576 Phase 2 Vorinostat;Bortezomib
14 Romidepsin, Ifosfamide, Carboplatin, and Etoposide in Treating Participants With Relapsed or Refractory Peripheral T-Cell Lymphoma Completed NCT01590732 Phase 1 Carboplatin;Etoposide;Ifosfamide;Romidepsin
15 Study of MLN8237 in Participants With Advanced Hematological Malignancies Completed NCT00697346 Phase 1 Alisertib
16 T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas Recruiting NCT03049449 Phase 1 Cyclophosphamide;Fludarabine
17 Everolimus and Bortezomib in Treating Patients With Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1 Bortezomib;Everolimus
18 Comparison Between Axial- and Lateral-viewing Capsule Endoscopy in Celiac Disease Unknown status NCT03095573 Not Applicable
19 Chemoimmunotherapy and Allogeneic Stem Cell Transplant for NK T-cell Leukemia/Lymphoma Recruiting NCT03719105 Early Phase 1 Methotrexate;pralatraxate,;Ifosfamide;Dexamethasone;Etoposide;Pegaspargase;cyclophosphamide;Doxorubicin;Prednisone;Brentuximab Vedotin
20 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409 Not Applicable
21 MAgnetic Resonance Imaging in COeliac Disease Active, not recruiting NCT02551289
22 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Active, not recruiting NCT02652715 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:

42
T Cells, Small Intestine, Testes, B Cells, Bone, Colon, Lymph Node

Publications for Enteropathy-Associated T-Cell Lymphoma

Articles related to Enteropathy-Associated T-Cell Lymphoma:

(show top 50) (show all 137)
# Title Authors Year
1
Hypocalcemia Revealing an Enteropathy-Associated T-cell Lymphoma. ( 30858951 )
2019
2
Intestinal T-cell lymphoma with enteropathy-associated T-cell lymphoma-like features arising in the setting of adult autoimmune enteropathy. ( 29446107 )
2018
3
Coexpression of CD3 and CD20 in Canine Enteropathy-Associated T-cell Lymphoma. ( 29343197 )
2018
4
Type II enteropathy-associated t-cell lymphoma: A case report and literature review. ( 29888733 )
2018
5
Pathogenesis of Enteropathy-Associated T Cell Lymphoma. ( 29943210 )
2018
6
An unexpected deterrent in diagnosing refractory celiac disease and enteropathy-associated T-cell lymphoma: a gluten-free diet. ( 30181834 )
2018
7
Perforation of the small intestine caused by enteropathy-associated T cell lymphoma. ( 30302434 )
2018
8
Potentially important miRNAs in enteropathy-associated T-cell lymphoma pathogenesis: A pilot study. ( 30989051 )
2018
9
A Case of Type II Enteropathy-Associated T-Cell Lymphoma in a Patient With Ulcerative Colitis. ( 28572650 )
2017
10
High myeloid-derived suppressor cell frequencies in the duodenum are associated with enteropathy associated T-cell lymphoma and its precursor lesions. ( 27341082 )
2017
11
Enteropathy-associated T-cell lymphoma presenting as cutaneous deposits. ( 27766624 )
2017
12
Establishment of a Patient-Derived Xenograft of Canine Enteropathy-Associated T-Cell Lymphoma, Large Cell Type. ( 28011041 )
2017
13
Type II Enteropathy-Associated T-cell Lymphoma: A Rare Report from Iran. ( 28316767 )
2017
14
Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2. ( 28424246 )
2017
15
Enteropathy-associated T-cell lymphoma in the US: higher incidence and poorer survival among Asians. ( 26104061 )
2016
16
Cross Lineage Rearrangement in Feline Enteropathy-Associated T-cell Lymphoma. ( 26215761 )
2016
17
Endoscopic Findings of Enteropathy-Associated T-Cell Lymphoma Type II: A Case Series. ( 26260757 )
2016
18
Type I enteropathy-associated T-cell lymphoma in the colon of a 29-year-old patient and a brief literature review. ( 26955284 )
2016
19
Eosinophil-Rich Acute Febrile Neutrophilic Dermatosis in a Patient With Enteropathy-Associated T-cell Lymphoma, Type 1. ( 27097333 )
2016
20
Enteropathy-associated T-cell lymphoma: An extremely rare cause of chronic diarrhoea. ( 27326953 )
2016
21
Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations. ( 27600764 )
2016
22
Enteropathy-Associated T-Cell Lymphoma. ( 27900603 )
2016
23
A rare but potentially fatal cause of diarrhoea and weight loss: enteropathy-associated T-cell lymphoma. ( 25568262 )
2015
24
Enteropathy-associated T-cell lymphoma in childhood: a case report and review of the literature. ( 25629982 )
2015
25
Treatment response in enteropathy associated T-cell lymphoma; survival in a large multicenter cohort. ( 25716069 )
2015
26
A New and Validated Clinical Prognostic Model (EPI) for Enteropathy-Associated T-cell Lymphoma. ( 25779949 )
2015
27
Enteropathy-associated T-cell lymphoma: improving treatment strategies. ( 25925928 )
2015
28
Primary Enteropathy-Associated T-Cell Lymphoma Type 2: An Emerging Entity? ( 26068772 )
2015
29
Enteropathy-associated T-cell lymphoma: involvement of the gastrointestinal tract from the duodenum to the rectum. ( 26115398 )
2015
30
Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan. ( 26226842 )
2015
31
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
32
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
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, type II (monomorphic variant). ( 24822244 )
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-monomorphic variant, presenting as bilateral ovarian masses. ( 24943780 )
2014
37
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
38
Profile of CD103 expression in T-cell neoplasms: immunoreactivity is not restricted to enteropathy-associated T-cell lymphoma. ( 25025448 )
2014
39
An unusual case of enteropathy-associated T-cell lymphoma with CD20 positivity. ( 25308040 )
2014
40
Enteropathy associated T cell lymphoma - a case report of an uncommon extranodal T cell lymphoma. ( 25478355 )
2014
41
Enteropathy-associated T cell lymphoma as a complication of silent celiac disease. ( 25568762 )
2014
42
A Case of Type II Enteropathy-Associated T-Cell Lymphoma with Epstein-Barr Virus Positivity. ( 25588633 )
2014
43
Hemophagocytic Lymphohistiocytic Syndrome and Enteropathy-Associated T-cell Lymphoma in a Patient with Refractory Celiac Disease. ( 26157854 )
2014
44
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
45
Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study. ( 23313469 )
2013
46
Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT. ( 23361910 )
2013
47
Brentuximab in the treatment of CD30-positive enteropathy-associated T-cell lymphoma. ( 23411380 )
2013
48
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
49
Hemophagocytic lymphohistiocytosis: an uncommon presentation of enteropathy-associated T-cell lymphoma. ( 23530098 )
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
12.48 CCL11 CD3G ITGAE
2 10.64 CD3G ITGAE
3 10.63 CD3G ITGAE

GO Terms for Enteropathy-Associated T-Cell Lymphoma

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 CD3G ITGAE

Sources for Enteropathy-Associated T-Cell Lymphoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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