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Enteropathy-Associated T-Cell Lymphoma

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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: 65 relevant articles (3 outcomes, 4 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	T-Cell Lymphoma (Lymphoma, T Cell)
2.	Lymphoma (Lymphomas)
3.	Anaplastic Large-Cell Lymphoma (Ki 1 Lymphoma)
4.	Peripheral T-Cell Lymphoma (Lymphoma, T Cell, Peripheral)
5.	Extranodal NK-T-Cell Lymphoma

Experts

1.	Mulder, Chris J J: 4 articles (01/2015 - 06/2005)
2.	Cellier, Christophe: 4 articles (01/2015 - 06/2005)
3.	Malamut, Georgia: 3 articles (01/2015 - 06/2010)
4.	Cerf-Bensussan, Nadine: 3 articles (01/2015 - 06/2010)
5.	Nijeboer, Petula: 2 articles (11/2015 - 01/2015)
6.	Bouma, Gerd: 2 articles (11/2015 - 01/2015)
7.	van Bergen, Jeroen: 2 articles (01/2015 - 12/2008)
8.	Koning, Frits: 2 articles (01/2015 - 12/2008)
9.	Duchosal, Michel A: 2 articles (08/2014 - 03/2013)
10.	Jaffe, Elaine S: 2 articles (01/2013 - 01/2009)

Drugs and Biologics

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

1.	Cladribine FDA LinkGeneric 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.	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. "
3.	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. " 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." 10/01/2011 - "Type II enteropathy-associated T-cell lymphoma: a distinct aggressive lymphoma with frequent γδ T-cell receptor expression."
4.	Subcutaneous panniculitis-like T-cell lymphomaIBA 12/05/2015 - "Subtypes, such as enteropathy-associated T-cell lymphoma, hepatosplenic T-cell lymphoma, extranodal NK/T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma, have an approximate annual incidence in United States of <500 each. " 01/01/2013 - "They can be derived from either αβ or γδ cytotoxic T cells, and include subcutaneous panniculitis-like T-cell lymphoma, and enteropathy-associated T-cell lymphomas, both Type I and Type II. Type I enteropathy-associated lymphomas occur in association with celiac disease, whereas Type II lymphomas are more often sporadic. " 01/01/2009 - "Among the peripheral T-cell lymphomas (PTCL), more precise definitions were introduced for several entities, including anaplastic large cell lymphoma, angioimmunoblastic T-cell lymphoma, enteropathy-associated T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma. " 11/01/2011 - "The basic categories include precursor lymphoid neoplasms (eg, lymphoblastic lymphoma); mature natural killer/T-cell neoplasms and extranodal lymphomas, including enteropathy-associated T-cell lymphoma; hepatosplenic T-cell lymphoma; and subcutaneous panniculitis-like T-cell lymphoma. " 10/01/2015 - "Of eligible 159 patients (113 males, 46 females), median age was 53 years old The histological subtypes included peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) 35.8%, angioimmunoblastic T-cell lymphoma (AITL) 18.2%, extranodal NK/T-cell lymphoma (ENKL) 17.0%, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) 12.6%, cutaneous T-cell lymphoma (CTCL) 11.3%, anaplastic large cell lymphoma (ALCL) 4.4%, and enteropathy-associated T-cell lymphoma (EATL) 0.6%. "
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). " 01/01/2013 - "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.	tranilast (N 5')IBA 11/21/2015 - "A total of 40 patients with (pre)malignant conditions of CD, ulcerative jejunitis (n = 5) and enteropathy associated T-cell lymphoma (EATL) (n = 35), who underwent surgery between 2002 and 2013 were retrospectively evaluated. " 03/01/2003 - "Histological subtypes were: nodular sclerosing HD (n = 10), diffuse large B cell (n = 5), T cell-rich B cell (n = 2), follicular (n = 2), mantle cell (n = 1) and enteropathy-associated T-cell lymphoma (n = 1). " 08/01/2014 - "Diagnoses included PTCL-not otherwise specified (n = 19), anaplastic large cell lymphoma (n = 11), angioimmunoblastic T-cell lymphoma (n = 5), enteropathy-associated T-cell lymphoma (n = 5) and other rare subtypes (n = 3). " 03/01/2011 - "The histological subtypes were as follows: peripheral T-cell lymphoma, not otherwise specified n=13; anaplastic large cell lymphoma (ALCL) ALK-negative n=5; ALCL ALK-positive n=3; ALCL with an unknown ALK status n=3; angioimmunoblastic lymphoma n=1; hepatosplenic lymphoma n=1; Sézary syndrome n=1; and enteropathy-associated T-cell lymphoma n=2. The median age at diagnosis was 48 years (29-64), most patients had advanced Ann Arbor stages (22 patients, 77%), IPI score ≥3 was found in 13 (45%) and PIT score ≥2 in 17 (59%) of the 29 patients. " 12/01/1992 - "According to the pattern of lymphoma infiltration and the morphology of the uninvolved small intestinal mucosa, 21 cases were separated histologically into three categories; 1) enteropathy-associated T-cell lymphoma (EATCL, n = 9) showing predominant intramucosal lymphoma spread and villous atrophy of uninvolved mucosa with high density of intraepithelial lymphocytes (IEL), 2) EATCL-like lymphoma without enteropathy (EATCL-LLWE, n = 5) but with an infiltration pattern similar to EATCL, and 3) T-cell lymphoma without features of EATCL (Non-EATCL, n = 7). "
9.	ParaffinIBA 01/15/1997 - "Paraffin-embedded tissue from 43 primary intestinal NHLs (19 cases from Mexico and 24 from Western Europe) were examined, including 17 high grade B-NHLs, 9 low grade B-NHLs, and 17 T-NHLs; 6 of which were enteropathy associated T-cell lymphomas. " 04/01/1999 - "We report a case of small-intestinal NK cell lymphoma that was originally diagnosed as an enteropathy-associated T-cell lymphoma based on paraffin immunohistochemistry. " 10/01/1997 - "The tumour tissue comprised two T cell subtypes of the one family, T cell receptor V beta 9. RNA and DNA were isolated from formalin fixed paraffin wax embedded enteropathy associated T cell lymphoma tissue. " 10/01/1997 - "To isolate RNA and DNA simultaneously from formalin fixed paraffin wax embedded tissue to assess the clonality of enteropathy associated T cell lymphomas and to analyse it in detail by a non-radioactive method of T cell receptor complementarity determining region 3 (CDR3) spectratyping. "
10.	Interleukin-15 (Interleukin 15)IBA 06/01/2010 - "Here we show that the survival signals delivered by IL-15 to freshly isolated human IELs and to human IEL cell lines derived from CD patients with type II refractory CD (RCDII) - a clinicopathological entity considered an intermediary step between CD and enteropathy-associated T cell lymphoma - depend on the antiapoptotic factors Bcl-2 and/or Bcl-xL. " 04/01/2006 - "IL-15 mucosal levels were also determined by ELISA in five patients with complicated CD (two ulcerative jejunoileites, one refractory sprue, and two enteropathy associated T cell lymphomas) tested for T cell receptor gamma chain clonality. "

Therapies and Procedures

1.	Stem Cell Transplantation 03/28/2013 - "Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT." 05/06/2010 - "Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation." 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." 12/01/2003 - "High-dose therapy supported by autologous stem cell transplantation in patients with enteropathy-associated T-cell lymphoma."
2.	Drug Therapy (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." 04/01/2012 - "Enteropathy-associated T-cell lymphoma is a rare form of T-cell lymphoma associated with a poor prognosis and the relative ineffectiveness of standard chemotherapy. "
3.	Transplants (Transplant) 06/01/2006 - "Successful treatment of enteropathy-associated T cell lymphoma with autologous stem cell transplant." 01/01/1992 - "Enteropathy-associated T-cell lymphoma in a renal transplant patient with evidence of Epstein-Barr virus involvement." 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."
4.	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."
5.	Kidney Transplantation 01/01/1992 - "The clinical and histological findings in a 54-year-old patient with enteropathy-associated T-cell lymphoma (EATL) occurring 18 years after renal transplantation are presented. "