HAEM5:Enteropathy-associated T-cell lymphoma: Difference between revisions
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*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. < | *Most of the EATL cases develop through an intermediate step of RCDII, however it can also arise de novo in patients with celiac disease.<ref name=":13">{{Cite journal|last=Cording|first=Sascha|last2=Lhermitte|first2=Ludovic|last3=Malamut|first3=Georgia|last4=Berrabah|first4=Sofia|last5=Trinquand|first5=Amélie|last6=Guegan|first6=Nicolas|last7=Villarese|first7=Patrick|last8=Kaltenbach|first8=Sophie|last9=Meresse|first9=Bertrand|date=2022-03|title=Oncogenetic landscape of lymphomagenesis in coeliac disease|url=https://pubmed.ncbi.nlm.nih.gov/33579790|journal=Gut|volume=71|issue=3|pages=497–508|doi=10.1136/gutjnl-2020-322935|issn=1468-3288|pmc=8862029|pmid=33579790}}</ref> | ||
*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> | *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><ref>{{Cite journal|last=Soderquist|first=Craig R.|last2=Bhagat|first2=Govind|date=2021|title=Cellular and molecular bases of refractory celiac disease|url=https://pubmed.ncbi.nlm.nih.gov/33707055|journal=International Review of Cell and Molecular Biology|volume=358|pages=207–240|doi=10.1016/bs.ircmb.2020.12.001|issn=1937-6448|pmid=33707055}}</ref>. | ||
*Celiac disease may be diagnosed prior to EATL diagnosis in 20-73% of cases, or both entities may be diagnosed concomitantly in 10-58% of the cases<ref name=":5" /> | *Celiac disease may be diagnosed prior to EATL diagnosis in 20-73% of cases, or both entities may be diagnosed concomitantly in 10-58% of the cases<ref name=":5" /> | ||
*Risk factors include homozygosity for HLA-DQ2 and advanced age<ref name=":5" /> | *Risk factors include homozygosity for HLA-DQ2 and advanced age<ref name=":5" /> | ||
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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 [23313469, 26462278] | *Approximately 25% of EATLs (primarily de novo EATL) are CD8+, and rare cases express TCRγδ [23313469, 26462278] | ||
*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. < | *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: | ||
Positive (universal) - CD3, CD7 | Positive (universal) - CD3, CD7 | ||