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

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<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}
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*Abdominal pain
*Abdominal pain
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*Long-standing history of malabsorption is atypical
*Long-standing history of malabsorption is atypical


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==Sites of Involvement==
==Sites of Involvement==
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<blockquote class='blockedit'>{{Box-round|title=v4:Immunophenotype|The content below was from the old template. Please incorporate above.}}
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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
'''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


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'''INCORPORATE INTO TABLE'''
'''INCORPORATE INTO TABLE'''


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==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
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*N/A
*N/A
*'''No consistent gene fusion reported'''
*'''No consistent gene fusion reported'''


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* Individual Region Genomic Gain/Loss/LOH
* Individual Region Genomic Gain/Loss/LOH
* Characteristic Chromosomal Patterns
* Characteristic Chromosomal Patterns
* Gene Mutations (SNV/INDEL)}}
* Gene Mutations (SNV/INDEL)}}</blockquote>


*'''Diagnosis'''
*'''Diagnosis'''
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**PEG-asparaginase has been considered as option<ref>{{Cite journal|last=C|first=Gentille|last2=Q|first2=Qin|last3=A|first3=Barbieri|last4=Ps|first4=Ravi|last5=S|first5=Iyer|date=2017|title=Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma, a disease with diagnostic and therapeutic challenges|url=https://pubmed.ncbi.nlm.nih.gov/29062389/|language=en|doi=10.3332/ecancer.2017.771|pmc=PMC5636209|pmid=29062389}}</ref>
**PEG-asparaginase has been considered as option<ref>{{Cite journal|last=C|first=Gentille|last2=Q|first2=Qin|last3=A|first3=Barbieri|last4=Ps|first4=Ravi|last5=S|first5=Iyer|date=2017|title=Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma, a disease with diagnostic and therapeutic challenges|url=https://pubmed.ncbi.nlm.nih.gov/29062389/|language=en|doi=10.3332/ecancer.2017.771|pmc=PMC5636209|pmid=29062389}}</ref>


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==Individual Region Genomic Gain / Loss / LOH==
==Individual Region Genomic Gain / Loss / LOH==
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<blockquote class='blockedit'>{{Box-round|title=v4:Genomic Gain/Loss/LOH|The content below was from the old template. Please incorporate above.}}
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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.}}<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>
<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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==Characteristic Chromosomal Patterns==
==Characteristic Chromosomal Patterns==
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<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}
<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}</blockquote>


*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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==Gene Mutations (SNV / INDEL)==
==Gene Mutations (SNV / INDEL)==
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<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}
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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.}}<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>
<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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==Epigenomic Alterations==
==Epigenomic Alterations==
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<blockquote class='blockedit'>{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}
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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.}}<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>
<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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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==