HAEM5:Enteropathy-associated T-cell lymphoma: Difference between revisions

 

|N/A

|Type I enteropathy-associated T-cell lymphoma

!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)

!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T

!Established Clinical Significance Per Guidelines - Yes or No (Source)

!Clinical Relevance Details/Other Notes

!Prevalence -  

Common >20%, Recurrent 5-20% or Rare <5% (Disease)

!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T

!Established Clinical Significance Per Guidelines - Yes or No (Source)

!Clinical Relevance Details/Other Notes

!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -

Common >20%, Recurrent 5-20% or Rare <5% (Disease)

!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T  

!Established Clinical Significance Per Guidelines - Yes or No (Source)

!Clinical Relevance Details/Other Notes

!Gene; Genetic Alteration<ref name=":4" /><ref>{{Cite journal|last=Sh|first=Swerdlow|last2=Jr|first2=Cook|date=2020|title=As the world turns, evolving lymphoma classifications-past, present and future|url=https://pubmed.ncbi.nlm.nih.gov/31493426/|language=en|pmid=31493426}}</ref>!!Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)!!Prevalence (COSMIC /  TCGA / Other)<ref name=":4" />!!Concomitant Mutations!!Mutually Exclusive Mutations

!Diagnostic Significance (Yes, No or Unknown)

|23% 48%

|16%, 38%

|SOCS3

|TSG

|8%

*Dissemination to extra gastrointestinal sites: mesenteric and abdominal lymph nodes > bone marrow, lung, liver or skin<ref name=":2" /><ref>{{Cite journal|last=Egan|first=L. J.|last2=Walsh|first2=S. V.|last3=Stevens|first3=F. M.|last4=Connolly|first4=C. E.|last5=Egan|first5=E. L.|last6=McCarthy|first6=C. F.|date=1995-09|title=Celiac-associated lymphoma. A single institution experience of 30 cases in the combination chemotherapy era|url=https://pubmed.ncbi.nlm.nih.gov/8583077|journal=Journal of Clinical Gastroenterology|volume=21|issue=2|pages=123–129|issn=0192-0790|pmid=8583077}}</ref><ref name=":14">{{Cite journal|last=Malamut|first=Georgia|last2=Chandesris|first2=Olivia|last3=Verkarre|first3=Virginie|last4=Meresse|first4=Bertrand|last5=Callens|first5=Céline|last6=Macintyre|first6=Elizabeth|last7=Bouhnik|first7=Yoram|last8=Gornet|first8=Jean-Marc|last9=Allez|first9=Matthieu|date=2013-05|title=Enteropathy associated T cell lymphoma in celiac disease: a large retrospective study|url=https://pubmed.ncbi.nlm.nih.gov/23313469|journal=Digestive and Liver Disease: Official Journal of the Italian Society of Gastroenterology and the Italian Association for the Study of the Liver|volume=45|issue=5|pages=377–384|doi=10.1016/j.dld.2012.12.001|issn=1878-3562|pmc=7185558|pmid=23313469}}</ref>.

*Approximately 25% of EATLs (primarily de novo EATL) are CD8+, and rare cases express TCRγδ<ref name=":14" /><ref>{{Cite journal|last=van Wanrooij|first=R. L. J.|last2=de Jong|first2=D.|last3=Langerak|first3=A. W.|last4=Ylstra|first4=B.|last5=van Essen|first5=H. F.|last6=Heideman|first6=D. a. M.|last7=Bontkes|first7=H. J.|last8=Mulder|first8=C. J. J.|last9=Bouma|first9=G.|date=2015|title=Novel variant of EATL evolving from mucosal γδ-T-cells in a patient with type I RCD|url=https://pubmed.ncbi.nlm.nih.gov/26462278|journal=BMJ open gastroenterology|volume=2|issue=1|pages=e000026|doi=10.1136/bmjgast-2014-000026|issn=2054-4774|pmc=4599158|pmid=26462278}}</ref>