HAEM5:Enteropathy-associated T-cell lymphoma: Difference between revisions
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==Definition / Description of Disease== | ==Definition / Description of Disease== | ||
*'''Enteropathy-associated T-cell lymphoma (EATL) is an aggressive intestinal T-cell lymphoma more common in patients with celiac disease, especially type II refractory celiac disease (RCDII).''' | *'''Enteropathy-associated T-cell lymphoma (EATL) is an aggressive intestinal T-cell lymphoma more common in patients with celiac disease, especially type II refractory celiac disease (RCDII).''' | ||
*'''Most of the EATL cases develop through an intermediate step of RCDII, however it can also arise de novo in patients with celiac disease. <u>33579790</u>''' | *'''Most of the EATL cases develop through an intermediate step of RCDII, however it can also arise de novo in patients with celiac disease. <u>33579790</u>''' | ||
*'''RCDII can be defined as failure to respond to a strict gluten-free diet for at least 12 months and is associated with clonal expansion of immunophenotypically an aberrant IELs''' <ref name=":5">Govind Bhagat, et al. Enteropathy-associated T-cell lymphoma. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet]. Lyon (France): International Agency for Research on Cancer; 2024 [cited 2024 July 2]. (WHO classification of tumors series, 5th ed.; vol. 11). Available from: <nowiki>https://tumourclassification.iarc.who.int/chaptercontent/63/224</nowiki> </ref>, { 33707055 }. | *'''RCDII can be defined as failure to respond to a strict gluten-free diet for at least 12 months and is associated with clonal expansion of immunophenotypically an aberrant IELs''' <ref name=":5">Govind Bhagat, et al. Enteropathy-associated T-cell lymphoma. In: WHO Classification of Tumours Editorial Board. Haematolymphoid tumours [Internet]. Lyon (France): International Agency for Research on Cancer; 2024 [cited 2024 July 2]. (WHO classification of tumors series, 5th ed.; vol. 11). Available from: <nowiki>https://tumourclassification.iarc.who.int/chaptercontent/63/224</nowiki> </ref>, { 33707055 }. | ||
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* '''CD can be diagnosed at the time of EATL diagnosis'''<ref name=":5" /> | *'''CD can be diagnosed at the time of EATL diagnosis'''<ref name=":5" /> | ||
* '''Patients with RCD can sometimes present with small-intestinal ulceration (ulcerative jejunitis) { 9250161 ; 10381521 }.''' | *'''Patients with RCD can sometimes present with small-intestinal ulceration (ulcerative jejunitis) { 9250161 ; 10381521 }.''' | ||
If there is no prior diagnosis of celiac disease and lymphoma is the initial presentation, the following findings can point towards celiac disease associated EATL: | If there is no prior diagnosis of celiac disease and lymphoma is the initial presentation, the following findings can point towards celiac disease associated EATL: | ||
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|JAK1 | |JAK1 | ||
|Oncogene | |Oncogene | ||
|23% | |23% '''48%''' | ||
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|STAT3 | |STAT3 | ||
|oncogene | |oncogene | ||
|16% | |16%, '''38%''' | ||
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|TSG | |TSG | ||
|7% | |7% | ||
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|'''SOCS3''' | |||
|'''TSG''' | |||
|'''8%''' | |||
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