HAEM5:Enteropathy-associated T-cell lymphoma: Difference between revisions
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*Small intestine (predominantly jejunum and ileum > large intestine and stomach)<ref name=":1" /> | *Small intestine (predominantly jejunum and ileum > large intestine and stomach)<ref name=":1" /> | ||
*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>{{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>. | *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>. | ||
*Metastases involve intra-abdominal node > bone marrow > lung > liver > skin<ref name=":1" /> | *Metastases involve intra-abdominal node > bone marrow > lung > liver > skin<ref name=":1" /> | ||
*CNS (rare)<ref name=":1" /> | *CNS (rare)<ref name=":1" /> | ||
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The <u>immunophenotype</u> of this disease is detailed below: | The <u>immunophenotype</u> of this disease is detailed below: | ||
*Approximately 25% of EATLs (primarily de novo EATL) are CD8+, and rare cases express TCRγδ | *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> | ||
*Ki-67 is very low and CD30 is negative in RCDII, their appearance is useful to monitor RCDII progression to EATL and to indicate the need for chemotherapeutic regimens targeting dividing cells, a therapeutic option that is inefficient and even dangerous in RCDII.<ref name=":13" /> | *Ki-67 is very low and CD30 is negative in RCDII, their appearance is useful to monitor RCDII progression to EATL and to indicate the need for chemotherapeutic regimens targeting dividing cells, a therapeutic option that is inefficient and even dangerous in RCDII.<ref name=":13" /> | ||
*The most common immunophenotypic profile in EATL is given below: | *The most common immunophenotypic profile in EATL is given below: | ||