Compendium of Cancer Genome Aberrations

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

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==Morphologic Features==
==Morphologic Features==


*Pleomorphic medium to large neoplastic lymphoid infiltrate<ref name=":2" />
*'''Pleomorphic medium to large sized neoplastic lymphoid cells with immunoblastic or anaplastic morphology.<ref name=":2" /><ref name=":5" />'''
*'''Angulated vesicular nuclei, prominent nucleoli, and a pale-staining cytoplasm [https://doi.org/10.3390/diagnostics13162629<nowiki>]</nowiki>'''
*'''Extensive inflammatory background of histiocytes, eosinophils, small lymphocytes, and plasma cells <ref name=":5" />'''
*'''Adjacent mucosa often displays the histological features of active CD, including increased IEL infiltration, crypt hyperplasia, and villous atrophy <ref name=":2" /><ref name=":5" /> [https://doi.org/10.3390/diagnostics13162629<nowiki>]</nowiki>'''
*'''Angioinvasion and angiodestruction are commonly seen''' <ref name=":5" />
*
*
*Neighborhood mucosa characterized by villous atrophy and crypt hyperplasia (non-malignant areas of celiac disease)<ref name=":2" />
*Neighborhood mucosa characterized by villous atrophy and crypt hyperplasia (non-malignant areas of celiac disease)<ref name=":2" />
*Round or angulated vesicular nuclei<ref name=":2" />
*Round or angulated vesicular nuclei<ref name=":2" />
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*Angiocentric and angioinvasive features with extensive necrosis<ref name=":2" />
*Angiocentric and angioinvasive features with extensive necrosis<ref name=":2" />


<br />
==Immunophenotype==
==Immunophenotype==


Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table'') </span>
* '''The most common immunophenotypic profile in EATL is given below:'''


{| class="wikitable sortable"
{| class="wikitable sortable"
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|}
|}


{| class="wikitable sortable"
* '''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. <u>33579790</u>'''
!Finding
 
!Type 1 RCD!!Type 2 RCD
{| class="wikitable"
|-
|+
|Positive (universal)
!
| ||CD3, CD7
!RCD Type 1
!RCD Type 2
!EATL
|-
|-
|Positive (frequent)
|'''Histopathology'''
| ||CD30 (harbinger of transformation to EATL from RCD2), NKP46 (not seen in IEL of CD or RCD1), CD103,
|Identical to uncomplicated CD
cytotoxic granule-associated markers (TIA1, granzyme B, perforin)
|Moderate/ severe villous atrophy with atypical IELs
|Infiltration of medium to large sized pleomorphic IELs
|-
|-
|Negative (frequent)
|'''IEL Immunophenotype'''
| ||CD4, CD8, CD5, CD56, TCR, EBER
|Like CD; sCD3+, CD8+
|
|
|-
|-
|Ki-67
|
| ||high
|
|
|
|}
|}
Immunophenotype of intraepithelial lymphocytes (IEL):<ref name=":7">{{Cite journal|last=P|first=Domizio|last2=Ra|first2=Owen|last3=Na|first3=Shepherd|last4=Ic|first4=Talbot|last5=Aj|first5=Norton|date=1993|title=Primary lymphoma of the small intestine. A clinicopathological study of 119 cases|url=https://pubmed.ncbi.nlm.nih.gov/8470758/|language=en|pmid=8470758}}</ref><ref name=":0">{{Cite journal|last=Deleeuw|first=Ronald J.|last2=Zettl|first2=Andreas|last3=Klinker|first3=Erdwine|last4=Haralambieva|first4=Eugenia|last5=Trottier|first5=Magan|last6=Chari|first6=Raj|last7=Ge|first7=Yong|last8=Gascoyne|first8=Randy D.|last9=Chott|first9=Andreas|date=2007-05|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|journal=Gastroenterology|volume=132|issue=5|pages=1902–1911|doi=10.1053/j.gastro.2007.03.036|issn=0016-5085|pmid=17484883}}</ref>
Immunophenotype of intraepithelial lymphocytes (IEL):<ref name=":7">{{Cite journal|last=P|first=Domizio|last2=Ra|first2=Owen|last3=Na|first3=Shepherd|last4=Ic|first4=Talbot|last5=Aj|first5=Norton|date=1993|title=Primary lymphoma of the small intestine. A clinicopathological study of 119 cases|url=https://pubmed.ncbi.nlm.nih.gov/8470758/|language=en|pmid=8470758}}</ref><ref name=":0">{{Cite journal|last=Deleeuw|first=Ronald J.|last2=Zettl|first2=Andreas|last3=Klinker|first3=Erdwine|last4=Haralambieva|first4=Eugenia|last5=Trottier|first5=Magan|last6=Chari|first6=Raj|last7=Ge|first7=Yong|last8=Gascoyne|first8=Randy D.|last9=Chott|first9=Andreas|date=2007-05|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|journal=Gastroenterology|volume=132|issue=5|pages=1902–1911|doi=10.1053/j.gastro.2007.03.036|issn=0016-5085|pmid=17484883}}</ref>


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****NKP46: significantly more positive in RCD2 IEL than normal IEL in CD and RCD1; not specific for RCD2 or EATL, can be seen in [[HAEM5:Monomorphic epitheliotropic intestinal T-cell lymphoma|MEITL]]; not seen in [[HAEM5:Indolent T-cell lymphoma of the gastrointestinal tract|indolent T-cell LPD of GI tract]]
****NKP46: significantly more positive in RCD2 IEL than normal IEL in CD and RCD1; not specific for RCD2 or EATL, can be seen in [[HAEM5:Monomorphic epitheliotropic intestinal T-cell lymphoma|MEITL]]; not seen in [[HAEM5:Indolent T-cell lymphoma of the gastrointestinal tract|indolent T-cell LPD of GI tract]]
****CD30+ indicates progression to EATL
****CD30+ indicates progression to EATL
**'''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. <u>33579790</u>'''


==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
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