Enteropathy-associated T-cell lymphoma pathophysiology: Difference between revisions
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*The [[JAK-STAT pathway]] is the most frequently mutated pathway. <ref name="pmid28424246">{{cite journal |vauthors=Moffitt AB, Ondrejka SL, McKinney M, Rempel RE, Goodlad JR, Teh CH, Leppa S, Mannisto S, Kovanen PE, Tse E, Au-Yeung RKH, Kwong YL, Srivastava G, Iqbal J, Yu J, Naresh K, Villa D, Gascoyne RD, Said J, Czader MB, Chadburn A, Richards KL, Rajagopalan D, Davis NS, Smith EC, Palus BC, Tzeng TJ, Healy JA, Lugar PL, Datta J, Love C, Levy S, Dunson DB, Zhuang Y, Hsi ED, Dave SS |title=Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2 |journal=J. Exp. Med. |volume=214 |issue=5 |pages=1371–1386 |date=May 2017 |pmid=28424246 |pmc=5413324 |doi=10.1084/jem.20160894 |url=}}</ref> | *The [[JAK-STAT pathway]] is the most frequently mutated pathway. <ref name="pmid28424246">{{cite journal |vauthors=Moffitt AB, Ondrejka SL, McKinney M, Rempel RE, Goodlad JR, Teh CH, Leppa S, Mannisto S, Kovanen PE, Tse E, Au-Yeung RKH, Kwong YL, Srivastava G, Iqbal J, Yu J, Naresh K, Villa D, Gascoyne RD, Said J, Czader MB, Chadburn A, Richards KL, Rajagopalan D, Davis NS, Smith EC, Palus BC, Tzeng TJ, Healy JA, Lugar PL, Datta J, Love C, Levy S, Dunson DB, Zhuang Y, Hsi ED, Dave SS |title=Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2 |journal=J. Exp. Med. |volume=214 |issue=5 |pages=1371–1386 |date=May 2017 |pmid=28424246 |pmc=5413324 |doi=10.1084/jem.20160894 |url=}}</ref> | ||
*[[Mutations]] in [[KRAS]], [[TP53]], and [[TERT]] Type I EATL and type II EATL (monomorphic intestinal T cell lymphoma) identified as well which have overlapping genetic alterations. | *[[Mutations]] in [[KRAS]], [[TP53]], and [[TERT]] Type I EATL and type II EATL (monomorphic intestinal T cell lymphoma) identified as well which have overlapping genetic alterations. | ||
==References== | |||
{{reflist|2}} | |||
Revision as of 19:12, 9 January 2019
Pathophysiology
- Enteropathy-associated T-cell Lymphoma (EATL), also enteropathy-type T-cell lymphoma (ETTL), is a type of T-cell lymphoma that affects the small intestine. [1]
- Frequent activating mutations in the JAK-STAT pathway in EATL suggests that deregulation of cytokine signaling is the early event in lymphomagenesis.
- Intraepithelial T cells are presumed to be the cell of origin of EATL (and RCD II).
- Variable degrees of transformations can be seen on histopathology of this tumor, but usually presents as large lymphoid cells.[2]
- These cancerous T-cells are a possible consequence of either refractory cases of celiac disease or chronic untreated celiac disease patients.
- Most commonly occurs in the jejunum or ileum.
- SETD2 was found to be the most often silenced gene in EATL according to studies.[3]
- The JAK-STAT pathway is the most frequently mutated pathway. [3]
- Mutations in KRAS, TP53, and TERT Type I EATL and type II EATL (monomorphic intestinal T cell lymphoma) identified as well which have overlapping genetic alterations.
References
- ↑ Bautista-Quach MA, Ake CD, Chen M, Wang J (September 2012). "Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features". J Gastrointest Oncol. 3 (3): 209–25. doi:10.3978/j.issn.2078-6891.2012.024. PMC 3418529. PMID 22943012.
- ↑ Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 27, 2016
- ↑ 3.0 3.1 Moffitt AB, Ondrejka SL, McKinney M, Rempel RE, Goodlad JR, Teh CH, Leppa S, Mannisto S, Kovanen PE, Tse E, Au-Yeung R, Kwong YL, Srivastava G, Iqbal J, Yu J, Naresh K, Villa D, Gascoyne RD, Said J, Czader MB, Chadburn A, Richards KL, Rajagopalan D, Davis NS, Smith EC, Palus BC, Tzeng TJ, Healy JA, Lugar PL, Datta J, Love C, Levy S, Dunson DB, Zhuang Y, Hsi ED, Dave SS (May 2017). "Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2". J. Exp. Med. 214 (5): 1371–1386. doi:10.1084/jem.20160894. PMC 5413324. PMID 28424246. Vancouver style error: initials (help)