Enteropathy-Associated T-Cell Lymphoma

A primary peripheral T-cell lymphoma in the gastrointestinal tract, most often in the jejunum, associated with a history of CELIAC DISEASE or other gastrointestinal diseases.

Also Known As:

Lymphoma, T-Cell, Enteropathy-Associated; T-Cell Lymphoma, Enteropathy-Associated; Enteropathy Associated T Cell Lymphoma; Enteropathy-Associated T-Cell Lymphomas; Lymphomas, Enteropathy-Associated T-Cell; T Cell Lymphoma, Enteropathy Associated; T-Cell Lymphomas, Enteropathy-Associated

Networked: 101 relevant articles (4 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Celiac Disease (Celiac Sprue)
2.	T-Cell Lymphoma (Lymphoma, T Cell)
3.	Lymphoma (Lymphomas)
4.	Anaplastic Large-Cell Lymphoma (Ki 1 Lymphoma)
5.	Peripheral T-Cell Lymphoma (Lymphoma, T Cell, Peripheral)

Experts

1.	Mulder, Chris J J: 6 articles (01/2021 - 06/2005)
2.	Mulder, C J J: 6 articles (01/2017 - 01/2007)
3.	Al-Toma, A: 4 articles (06/2011 - 02/2007)
4.	Verbeek, W H M: 4 articles (06/2011 - 01/2007)
5.	Schreurs, Marco W J: 3 articles (01/2021 - 12/2008)
6.	Cellier, Christophe: 3 articles (01/2015 - 06/2005)
7.	Brousse, N: 3 articles (04/2013 - 08/2001)
8.	Foss, Francine: 2 articles (01/2022 - 11/2017)
9.	Sokol, Lubomir: 2 articles (01/2022 - 11/2017)
10.	Di Sabatino, Antonio: 2 articles (05/2021 - 07/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Enteropathy-Associated T-Cell Lymphoma:

1.	Cladribine FDA LinkGeneric 01/01/2019 - "A 65-year-old male presented a monomorphic epitheliotropic intestinal T-cell lymphoma (formerly enteropathy-associated T-cell lymphoma, type II) with HCL not responding to first-line induction with cladribine. " 01/01/2015 - "Although cladribine with or without autologous stem cell transplantation is effective in the treatment of signs and symptoms of RCDII and improves survival as compared to symptomatic topical steroid therapy, cladribine failures still bear a high risk of malignant transformation, and the rate of enteropathy-associated T-cell lymphoma (EATL) development in this subgroup is extremely high. "
2.	SteroidsIBA 03/01/2002 - "After the exclusion of gluten in the diet, ulcerative jejunititis, and an enteropathy-associated T-cell lymphoma, another treatment modalities, such as systemic steroids and immunosuppressives, may be necessary. " 01/01/2015 - "Although cladribine with or without autologous stem cell transplantation is effective in the treatment of signs and symptoms of RCDII and improves survival as compared to symptomatic topical steroid therapy, cladribine failures still bear a high risk of malignant transformation, and the rate of enteropathy-associated T-cell lymphoma (EATL) development in this subgroup is extremely high. "
3.	GliadinIBA 10/11/1986 - "In contrast, none of 16 patients with enteropathy associated T cell lymphoma had raised levels of alpha gliadin antibody, and treatment with a gluten free diet resulted in histological improvement in one and transient clinical improvement in six patients. " 10/11/1986 - "Thus patients with enteropathy associated T cell lymphoma do not display a humoral immune response to wheat protein (alpha gliadin), rarely respond to a gluten free diet, and are often men. "
4.	T-Cell Antigen Receptors (T-Cell Receptor)IBA 04/01/2010 - "The diagnostic and follow-up biopsies from 33 patients with CD, 7 with suspected RCD, 41 with RCD and 20 with enteropathy-associated T cell lymphoma (EATL) (including 11 evolved from RCD) were investigated by CD3epsilon/CD8 double immunohistochemistry and PCR-based clonality analysis of the rearranged T cell receptor (TCR) genes. " 12/15/2008 - "Defective synthesis or association of T-cell receptor chains underlies loss of surface T-cell receptor-CD3 expression in enteropathy-associated T-cell lymphoma." 06/01/2005 - "In a subgroup of patients with RCD, an abnormal intraepithelial lymphocyte (IEL) population may be observed with the lack of surface expression of usual T-cell markers (CD3-CD8 and/or the T-cell receptor (TCR)) on IELs associated with T-cell clonality pattern suggest the presence of an early enteropathy-associated T-cell lymphoma (EATL) in a subgroup of patients with RCD. " 11/01/2022 - "BEST PRACTICE ADVICE 4: Use flow cytometry, immunohistochemistry, and T-cell receptor rearrangement studies to distinguish between subtypes of refractory celiac disease and to exclude enteropathy-associated T-cell lymphoma. " 06/01/1997 - "Enteropathy associated T cell lymphoma presenting as an isolated CNS lymphoma three years after diagnosis of coeliac disease: T cell receptor polymerase chain reaction studies failed to show the original enteropathy to be a clonal disorder."
5.	TrypsinIBA 06/01/1992 - "We have performed an immunohistochemical study of immunoblastic T cell lymphoma and enteropathy-associated T cell lymphoma for alpha-1 anti-trypsin and CD30. "
6.	HLA-DQ Antigens (HLA-DQ)IBA 03/01/2006 - "The aim of this study was to determine if a particular HLA-DQ subtype predisposes to complications such as refractory CD with (RCD II) or without aberrant T cells (RCD I), and enteropathy-associated T-cell lymphomas (EATL). "
7.	Glutens (Glutelin)IBA 04/01/2013 - "The dietary consumption of gluten-rich cereals triggers a gluten-specific immune response in genetically susceptible individuals leading to a spectrum of clinical manifestations ranging from an inapparent subclinical disease, to overt enteropathy that can in some individuals progress to enteropathy-associated T cell lymphoma (EATL). " 02/11/2013 - "This is the first report on contrast enhanced ultrasound (CEUS) findings in enteropathy associated T-cell lymphoma (EATL) complicating CMLNS in a gluten-free compliant patient with persistent symptoms and poor outcome. " 04/01/2008 - "We followed 1,757 celiac patients for a total period of 31,801 person-years, collecting data about the frequency of gluten intake and the incidence of the enteropathy-associated T-cell lymphoma. " 05/01/2004 - "It is well established that enteropathy associated T-cell lymphoma is associated with malabsorption which is due to gluten sensitivity (coeliac disease). " 03/01/1996 - "Similarly, enteropathy-associated T-cell lymphomas appear to result from a disordered response to gluten, although this profile remains controversial. "
8.	Anaplastic Lymphoma KinaseIBA 09/04/2014 - "In an intention-to-treat analysis in 252 nodal PTCL and enteropathy-associated T-cell lymphoma patients (excluding anaplastic lymphoma kinase-positive anaplastic large cell lymphoma), upfront auto-SCT was associated with a superior OS (HR, 0.58; P = .004) and PFS (HR, 0.56; P = .002) compared with patients treated without auto-SCT. " 01/01/2022 - "PTCL-not otherwise specified is the most common subtype, followed by angioimmunoblastic T-cell lymphoma, anaplastic large cell lymphoma, anaplastic lymphoma kinase-positive, anaplastic large cell lymphoma, anaplastic lymphoma kinase-negative, and enteropathy-associated T-cell lymphoma. " 01/01/2023 - "Lymphoma histology included PTCL not otherwise specified (n = 24), anaplastic lymphoma kinase-negative anaplastic large cell lymphoma (n = 14), angioimmunoblastic T cell lymphoma (n = 7), enteropathy-associated T cell lymphoma (n = 6), hepatosplenic T cell lymphoma (n = 4), and others (n = 10). " 01/01/2021 - "Auto-HCT is currently recommended to be used as frontline consolidation in peripheral T cell lymphoma not otherwise specified, angioimmunoblastic T cell lymphoma, anaplastic large cell lymphoma-anaplastic lymphoma kinase negative, NK/T cell (disseminated), and enteropathy-associated T cell lymphoma. " 05/01/2018 - "Participants were patients aged 18 years and older with bulky (tumour mass diameter >10 cm) stage I to stage IV disease (WHO performance status 0-3), previously untreated peripheral T-cell lymphoma not otherwise specified, angioimmunoblastic T-cell lymphoma, anaplastic lymphoma kinase-negative anaplastic large cell lymphoma, enteropathy-associated T-cell lymphoma, or hepatosplenic γδ T-cell lymphoma. "
9.	5- (benzoylamino)- N- (4- chlorophenyl)- 3- methyl- 4- isothiazolecarboxamide (ITCL)IBA 01/01/2022 - "Human enteropathy-associated T-cell lymphoma (EATL) is considered to be derived from intraepithelial lymphocytes (IELs); however, the origin of canine intestinal T-cell lymphoma (ITCL) remains unclear. " 07/01/2010 - "This series of six intestinal T cell lymphomas (ITCL) attempts to document enteropathy-associated T cell lymphoma (EATCL) in India. " 05/13/2023 - "A total of 28 patients were included: 17 enteropathy-associated T-cell lymphomas (EATL), 5 monomorphic epitheliotropic T-cell lymphomas (MEITL), 3 indolent T-cell lymphoproliferative disorders of the gastrointestinal tract (ITCLDGT), and 3 intestinal T-cell lymphomas not otherwise specified (ITCL-NOS). " 12/01/2022 - "Primary intestinal T-cell lymphomas (PITLs) comprise enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), extranodal NK/T-cell lymphoma (ENKTL), anaplastic large cell lymphoma (ALCL), and intestinal T cell lymphoma, NOS (ITCL-NOS). " 01/01/2022 - "Primary intestinal T-cell lymphoma (PITL) is highly aggressive and includes celiac disease-related enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), and primary intestinal peripheral T-cell lymphoma, not otherwise specified (ITCL-NOS). "
10.	Capsules (Microcapsules)IBA 08/01/2012 - "Enteropathy-associated T-cell lymphoma in small intestine detected by capsule endoscopy." 11/01/2022 - "BEST PRACTICE ADVICE 5: Perform small bowel imaging with capsule endoscopy and computed tomography or magnetic resonance enterography to exclude enteropathy-associated T-cell lymphoma and ulcerative jejunoileitis at initial diagnosis of type 2 refractory celiac disease. " 08/01/2013 - "Obscure gastrointestinal bleeding and video capsule retention due to enteropathy-associated T-cell lymphoma." 06/01/2012 - "The introduction of wireless video capsule endoscopy (VCE), small bowel enteroscopy and in particular double balloon enteroscopy (DBE), have expedited the accurate diagnosis of coeliac disease and its more serious complications such as small bowel adenocarcinoma, refractory coeliac disease type II (RCDII) and enteropathy associated T cell lymphoma (EATL)."

Therapies and Procedures

1.	Gluten-Free Diet 04/01/2019 - "Despite adherence to a gluten-free diet, there was no clinical remission and enteropathy-associated T cell lymphoma was suspected. " 05/01/2008 - "A complication of CD is the loss of response to a gluten-free diet called RCD that may progress to an enteropathy-associated T-cell lymphoma. " 04/01/2008 - "These results show that a strict gluten-free diet is protective towards the development of enteropathy-associated T-cell lymphoma." 06/01/2005 - "Loss of response to a gluten free diet (refractory coeliac disease) and ulcerative jejunitis are two recently described complications of CD that may progress to an Enteropathy-Associated T-cell Lymphoma (EATL). " 05/01/2005 - "We suggest that patients with coeliac disease unresponsive to a gluten free diet or with deteriorating clinical condition be investigated for complications such as enteropathy associated T-cell lymphoma. "
2.	Stem Cell Transplantation 03/28/2013 - "Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT." 07/01/2007 - "Autologous stem cell transplantation seems unsatisfactory for patients with enteropathy-associated T-cell lymphoma. " 07/01/2007 - "Disappointing outcome of autologous stem cell transplantation for enteropathy-associated T-cell lymphoma." 07/01/2007 - "Here we report on the feasibility, safety and efficacy of high-dose chemotherapy followed by autologous stem cell transplantation in patients with enteropathy-associated T-cell lymphoma (three upfront and one at relapse), with or without prior partial small bowel resection. " 05/06/2010 - "Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation."
3.	Therapeutics 06/01/2011 - "With current therapies, progression into enteropathy-associated T-cell lymphoma (EATL) occurs in 60-80% patients, with a high mortality rate. " 10/01/2007 - "RCD II is usually resistant to any known therapy and transition into enteropathy-associated T-cell lymphoma (EATL) is common. " 05/06/2010 - "Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation." 12/01/2003 - "High-dose therapy supported by autologous stem cell transplantation in patients with enteropathy-associated T-cell lymphoma." 08/01/2013 - "Great complications such as enteropathy-associated T-cell lymphoma may occur in a subgroup of these patients mainly in refractory celiac disease type 2. Curative therapies are still lacking. "
4.	Drug Therapy (Chemotherapy) 01/01/2023 - "This is a rare case of a patient with an enteropathy-associated T-cell lymphoma of gastric localisation, who developed a spontaneous gastric perforation in the absence of chemotherapy. " 04/01/2012 - "Enteropathy-associated T-cell lymphoma is associated with a poor outcome, independently of the chemotherapy regimens administered and frequent occurrence of haemophagocytic lymphohistiocytosis. " 01/01/2012 - "Second line chemotherapy in patients with enteropathy-associated T cell lymphoma: a retrospective single center analysis." 07/01/2007 - "Here we report on the feasibility, safety and efficacy of high-dose chemotherapy followed by autologous stem cell transplantation in patients with enteropathy-associated T-cell lymphoma (three upfront and one at relapse), with or without prior partial small bowel resection. " 01/01/2010 - "Successful treatment of refractory enteropathy-associated T-cell lymphoma using high-dose chemotherapy and autologous stem cell transplantation."
5.	Combination Drug Therapy (Combination Chemotherapy) 04/01/2007 - "Combination chemotherapy followed by autologous stem cell transplant for enteropathy-associated T-cell lymphoma." 01/01/2007 - "Combination chemotherapy followed by autologous stem cell transplant for enteropathy-associated T cell lymphoma."