HAEM5:Monomorphic epitheliotropic intestinal T-cell lymphoma: Difference between revisions

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{{DISPLAYTITLE:Monomorphic epitheliotropic intestinal T-cell lymphoma}}
{{DISPLAYTITLE:Monomorphic epitheliotropic intestinal T-cell lymphoma}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]


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{{Under Construction}}


<blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Monomorphic Epitheliotropic Intestinal T-cell Lymphoma]].
<blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Monomorphic Epitheliotropic Intestinal T-cell Lymphoma]].
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==Definition / Description of Disease==
*Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a primary intestinal T-cell lymphoma derived from intraepithelial lymphocytes that, unlike enteropathy-associated T-cell lymphoma, is not clearly associated with celiac disease
==Synonyms / Terminology==
*Formerly and no longer referred to as type II enteropathy-associated T-cell lymphoma (EATL)
==Epidemiology / Prevalence==
*More prevalent in Asian and Hispanic/indigenous population
*< 1 per 1,000,000
==Clinical Features==
Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
<span class="blue-text">EXAMPLE:</span> Fatigue
<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias
<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}
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*Abdominal pain
*Weight loss
*Diarrhea
*Long-standing history of malabsorption is atypical
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==Sites of Involvement==
*Small Intestine (jejunum > ileum) > large intestine > stomach
==Morphologic Features==
*Monomorphic small- to medium-sized neoplastic cells
*Uniformly round and regular nuclei
*Finely dispersed chromatin
*Inconspicuous nucleoli
*Abundant rim of pale cytoplasm
==Immunophenotype==
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{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||<span class="blue-text">EXAMPLE:</span> CD1
|-
|Positive (subset)||<span class="blue-text">EXAMPLE:</span> CD2
|-
|Negative (universal)||<span class="blue-text">EXAMPLE:</span> CD3
|-
|Negative (subset)||<span class="blue-text">EXAMPLE:</span> CD4
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'''Postive''': '''CD3, CD8, CD56''', TCR gamma > TCR beta, '''TIA1''', CD20 in 20% of cases, '''MATK''' in >80% of neoplastic cells helps distinguish from EATL, '''SYK''' <ref name=":1" /> (distinguishes from EATL), NKP46
Variably positive between cases: granzyme B, perforin
Negative: CD5, EBV/EBER
'''INCORPORATE INTO TABLE'''
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==WHO Essential and Desirable Genetic Diagnostic Criteria==
==WHO Essential and Desirable Genetic Diagnostic Criteria==
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
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*N/A
*N/A
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<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
<blockquote class="blockedit">{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
* Chromosomal Rearrangements (Gene Fusions)
* Chromosomal Rearrangements (Gene Fusions)
* Individual Region Genomic Gain/Loss/LOH
* Individual Region Genomic Gain/Loss/LOH
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<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref>{{Cite journal|last=Rj|first=Deleeuw|last2=A|first2=Zettl|last3=E|first3=Klinker|last4=E|first4=Haralambieva|last5=M|first5=Trottier|last6=R|first6=Chari|last7=Y|first7=Ge|last8=Rd|first8=Gascoyne|last9=A|first9=Chott|date=2007|title=Whole-genome analysis and HLA genotyping of enteropathy-type T-cell lymphoma reveals 2 distinct lymphoma subtypes|url=https://pubmed.ncbi.nlm.nih.gov/17484883/|language=en|pmid=17484883}}</ref><ref name=":3">{{Cite journal|last=S|first=Tomita|last2=Yy|first2=Kikuti|last3=J|first3=Carreras|last4=M|first4=Kojima|last5=K|first5=Ando|last6=H|first6=Takasaki|last7=R|first7=Sakai|last8=K|first8=Takata|last9=T|first9=Yoshino|date=2015|title=Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan|url=https://pubmed.ncbi.nlm.nih.gov/26226842/|language=en|pmid=26226842}}</ref><blockquote class="blockedit">
<blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref>{{Cite journal|last=Rj|first=Deleeuw|last2=A|first2=Zettl|last3=E|first3=Klinker|last4=E|first4=Haralambieva|last5=M|first5=Trottier|last6=R|first6=Chari|last7=Y|first7=Ge|last8=Rd|first8=Gascoyne|last9=A|first9=Chott|date=2007|title=Whole-genome analysis and HLA genotyping of enteropathy-type T-cell lymphoma reveals 2 distinct lymphoma subtypes|url=https://pubmed.ncbi.nlm.nih.gov/17484883/|language=en|pmid=17484883}}</ref><ref name=":3">{{Cite journal|last=S|first=Tomita|last2=Yy|first2=Kikuti|last3=J|first3=Carreras|last4=M|first4=Kojima|last5=K|first5=Ando|last6=H|first6=Takasaki|last7=R|first7=Sakai|last8=K|first8=Takata|last9=T|first9=Yoshino|date=2015|title=Genomic and immunohistochemical profiles of enteropathy-associated T-cell lymphoma in Japan|url=https://pubmed.ncbi.nlm.nih.gov/26226842/|language=en|pmid=26226842}}</ref><blockquote class="blockedit">
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*No pathognomonic aberrations/patterns described, but multiple genomic gains and losses are frequent
*No pathognomonic aberrations/patterns described, but multiple genomic gains and losses are frequent
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|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}</blockquote>
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<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0">{{Cite journal|last=Ab|first=Moffitt|last2=Sl|first2=Ondrejka|last3=M|first3=McKinney|last4=Re|first4=Rempel|last5=Jr|first5=Goodlad|last6=Ch|first6=Teh|last7=S|first7=Leppa|last8=S|first8=Mannisto|last9=Pe|first9=Kovanen|date=2017|title=Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2|url=https://pubmed.ncbi.nlm.nih.gov/28424246/|language=en|doi=10.1084/jem.20160894|pmc=PMC5413324|pmid=28424246}}</ref><ref name=":2">{{Cite journal|last=A|first=Roberti|last2=Mp|first2=Dobay|last3=B|first3=Bisig|last4=D|first4=Vallois|last5=C|first5=Boéchat|last6=E|first6=Lanitis|last7=B|first7=Bouchindhomme|last8=Mc|first8=Parrens|last9=C|first9=Bossard|date=2016|title=Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations|url=https://pubmed.ncbi.nlm.nih.gov/27600764/|language=en|doi=10.1038/ncomms12602|pmc=PMC5023950|pmid=27600764}}</ref><ref>{{Cite journal|last=Ml|first=Nairismägi|last2=J|first2=Tan|last3=Jq|first3=Lim|last4=S|first4=Nagarajan|last5=Cc|first5=Ng|last6=V|first6=Rajasegaran|last7=D|first7=Huang|last8=Wk|first8=Lim|last9=Y|first9=Laurensia|date=2016|title=JAK-STAT and G-protein-coupled receptor signaling pathways are frequently altered in epitheliotropic intestinal T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/26854024/|language=en|doi=10.1038/leu.2016.13|pmc=PMC4895162|pmid=26854024}}</ref><blockquote class="blockedit">
<blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0">{{Cite journal|last=Ab|first=Moffitt|last2=Sl|first2=Ondrejka|last3=M|first3=McKinney|last4=Re|first4=Rempel|last5=Jr|first5=Goodlad|last6=Ch|first6=Teh|last7=S|first7=Leppa|last8=S|first8=Mannisto|last9=Pe|first9=Kovanen|date=2017|title=Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2|url=https://pubmed.ncbi.nlm.nih.gov/28424246/|language=en|doi=10.1084/jem.20160894|pmc=PMC5413324|pmid=28424246}}</ref><ref name=":2">{{Cite journal|last=A|first=Roberti|last2=Mp|first2=Dobay|last3=B|first3=Bisig|last4=D|first4=Vallois|last5=C|first5=Boéchat|last6=E|first6=Lanitis|last7=B|first7=Bouchindhomme|last8=Mc|first8=Parrens|last9=C|first9=Bossard|date=2016|title=Type II enteropathy-associated T-cell lymphoma features a unique genomic profile with highly recurrent SETD2 alterations|url=https://pubmed.ncbi.nlm.nih.gov/27600764/|language=en|doi=10.1038/ncomms12602|pmc=PMC5023950|pmid=27600764}}</ref><ref>{{Cite journal|last=Ml|first=Nairismägi|last2=J|first2=Tan|last3=Jq|first3=Lim|last4=S|first4=Nagarajan|last5=Cc|first5=Ng|last6=V|first6=Rajasegaran|last7=D|first7=Huang|last8=Wk|first8=Lim|last9=Y|first9=Laurensia|date=2016|title=JAK-STAT and G-protein-coupled receptor signaling pathways are frequently altered in epitheliotropic intestinal T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/26854024/|language=en|doi=10.1038/leu.2016.13|pmc=PMC4895162|pmid=26854024}}</ref><blockquote class="blockedit">
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<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0" /><ref>{{Cite journal|last=A|first=Nicolae|last2=L|first2=Xi|last3=Th|first3=Pham|last4=Ta|first4=Pham|last5=W|first5=Navarro|last6=Hg|first6=Meeker|last7=S|first7=Pittaluga|last8=Es|first8=Jaffe|last9=M|first9=Raffeld|date=2016|title=Mutations in the JAK/STAT and RAS signaling pathways are common in intestinal T-cell lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/27389054/|language=en|doi=10.1038/leu.2016.178|pmc=PMC5093023|pmid=27389054}}</ref><blockquote class="blockedit">
<blockquote class="blockedit">{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}</blockquote><ref name=":0" /><ref>{{Cite journal|last=A|first=Nicolae|last2=L|first2=Xi|last3=Th|first3=Pham|last4=Ta|first4=Pham|last5=W|first5=Navarro|last6=Hg|first6=Meeker|last7=S|first7=Pittaluga|last8=Es|first8=Jaffe|last9=M|first9=Raffeld|date=2016|title=Mutations in the JAK/STAT and RAS signaling pathways are common in intestinal T-cell lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/27389054/|language=en|doi=10.1038/leu.2016.178|pmc=PMC5093023|pmid=27389054}}</ref><blockquote class="blockedit">
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(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references />
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==Notes==
==Notes==
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<nowiki>*</nowiki>''Citation of this Page'': “Monomorphic epitheliotropic intestinal T-cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Monomorphic_epitheliotropic_intestinal_T-cell_lymphoma</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Monomorphic epitheliotropic intestinal T-cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Monomorphic_epitheliotropic_intestinal_T-cell_lymphoma</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases M]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases M]]