Compendium of Cancer Genome Aberrations

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

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==Cancer Category / Type==
==Cancer Category / Type==


*HAEM5: Mature T- and NK-cell Neoplasms
*HAEM5: Mature T-cell and NK-cell neoplasms


==Cancer Sub-Classification / Subtype==
==Cancer Sub-Classification / Subtype==
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==Definition / Description of Disease==
==Definition / Description of Disease==


*'''EATL accounts for 3% of peripheral T-cell lymphomas and 66% of all primary intestinal T-cell lymphomas reported in Europe and the USA'''
*'''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).''' <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>
*'''Most of the EATL cases develop through an intermediate step of RCDII, however it can also arise de novo in patients with celiac disease. <u>33579790</u>'''
*'''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>, { 33707055 }.
*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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*0.5-1 in 1 million general population (2-5% in patients with celiac disease, 60-80% in patients with refractory celiac disease type 2)<ref name=":6">{{Cite journal|last=Wh|first=Verbeek|last2=Jm|first2=Van De Water|last3=A|first3=Al-Toma|last4=Jj|first4=Oudejans|last5=Cj|first5=Mulder|last6=Vm|first6=Coupé|date=2008|title=Incidence of enteropathy--associated T-cell lymphoma: a nation-wide study of a population-based registry in The Netherlands|url=https://pubmed.ncbi.nlm.nih.gov/18618372/|language=en|pmid=18618372}}</ref><ref name=":1">{{Cite journal|last=Aj|first=Ferreri|last2=Pl|first2=Zinzani|last3=S|first3=Govi|last4=Sa|first4=Pileri|date=2011|title=Enteropathy-associated T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/20655757/|language=en|pmid=20655757}}</ref><ref name=":2">{{Cite journal|last=J|first=Delabie|last2=H|first2=Holte|last3=Jm|first3=Vose|last4=F|first4=Ullrich|last5=Es|first5=Jaffe|last6=Kj|first6=Savage|last7=Jm|first7=Connors|last8=L|first8=Rimsza|last9=Nl|first9=Harris|date=2011|title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the international peripheral T-cell lymphoma project|url=https://pubmed.ncbi.nlm.nih.gov/21566094/|language=en|pmid=21566094}}</ref><ref>{{Cite journal|last=A|first=Rubio-Tapia|last2=Ja|first2=Murray|date=2010|title=Classification and management of refractory coeliac disease|url=https://pubmed.ncbi.nlm.nih.gov/20332526/|language=en|doi=10.1136/gut.2009.195131|pmc=PMC2861306|pmid=20332526}}</ref><ref>{{Cite journal|last=G|first=Malamut|last2=P|first2=Afchain|last3=V|first3=Verkarre|last4=T|first4=Lecomte|last5=A|first5=Amiot|last6=D|first6=Damotte|last7=Y|first7=Bouhnik|last8=Jf|first8=Colombel|last9=Jc|first9=Delchier|date=2009|title=Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II|url=https://pubmed.ncbi.nlm.nih.gov/19014942/|language=en|pmid=19014942}}</ref>
*0.5-1 in 1 million general population (2-5% in patients with celiac disease, 60-80% in patients with refractory celiac disease type 2)<ref name=":6">{{Cite journal|last=Wh|first=Verbeek|last2=Jm|first2=Van De Water|last3=A|first3=Al-Toma|last4=Jj|first4=Oudejans|last5=Cj|first5=Mulder|last6=Vm|first6=Coupé|date=2008|title=Incidence of enteropathy--associated T-cell lymphoma: a nation-wide study of a population-based registry in The Netherlands|url=https://pubmed.ncbi.nlm.nih.gov/18618372/|language=en|pmid=18618372}}</ref><ref name=":1">{{Cite journal|last=Aj|first=Ferreri|last2=Pl|first2=Zinzani|last3=S|first3=Govi|last4=Sa|first4=Pileri|date=2011|title=Enteropathy-associated T-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/20655757/|language=en|pmid=20655757}}</ref><ref name=":2">{{Cite journal|last=J|first=Delabie|last2=H|first2=Holte|last3=Jm|first3=Vose|last4=F|first4=Ullrich|last5=Es|first5=Jaffe|last6=Kj|first6=Savage|last7=Jm|first7=Connors|last8=L|first8=Rimsza|last9=Nl|first9=Harris|date=2011|title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the international peripheral T-cell lymphoma project|url=https://pubmed.ncbi.nlm.nih.gov/21566094/|language=en|pmid=21566094}}</ref><ref>{{Cite journal|last=A|first=Rubio-Tapia|last2=Ja|first2=Murray|date=2010|title=Classification and management of refractory coeliac disease|url=https://pubmed.ncbi.nlm.nih.gov/20332526/|language=en|doi=10.1136/gut.2009.195131|pmc=PMC2861306|pmid=20332526}}</ref><ref>{{Cite journal|last=G|first=Malamut|last2=P|first2=Afchain|last3=V|first3=Verkarre|last4=T|first4=Lecomte|last5=A|first5=Amiot|last6=D|first6=Damotte|last7=Y|first7=Bouhnik|last8=Jf|first8=Colombel|last9=Jc|first9=Delchier|date=2009|title=Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II|url=https://pubmed.ncbi.nlm.nih.gov/19014942/|language=en|pmid=19014942}}</ref>
*'''More common in regions with a high seroprevalence of CD, particularly Europe (0.05–0.14 cases per 100 000 population) { 15825131 ; 20197551 ; 18618372 } and the USA (0.016 cases per 100 000 population)'''
*'''EATL accounts for 3% of peripheral T-cell lymphomas and represents 66% of all primary intestinal T-cell lymphomas'''<ref name=":5" />
*'''More common in regions with a high prevalence of CD, particularly Europe (0.05–0.14 cases per 100 000 population) { 15825131 ; 20197551 ; 18618372 } and the USA (0.016 cases per 100 000 population)'''
*'''Extremely rare in Asia due to low population frequency of celiac HLA risk alleles<ref name=":6" /><ref name=":1" /><ref name=":2" />'''
*> 60% of all cases in intestinal T- cell lymphomas<ref name=":6" /><ref name=":1" /><ref name=":2" />
*> 60% of all cases in intestinal T- cell lymphomas<ref name=":6" /><ref name=":1" /><ref name=":2" />
*M:F 1.04:1 to 2.8:1<ref name=":6" /><ref name=":1" /><ref name=":2" />
*'''M:F 1.04:1 to 2.8:1'''<ref name=":6" /><ref name=":1" /><ref name=":2" />
*6th-7th decade of life<ref name=":6" /><ref name=":1" /><ref name=":2" />
*'''6th-7th decade of life'''<ref name=":6" /><ref name=":1" /><ref name=":2" />
*Mostly Caucasian (> 90%)<ref name=":6" /><ref name=":1" /><ref name=":2" />
*Mostly Caucasian (> 90%)<ref name=":6" /><ref name=":1" /><ref name=":2" />
*Uncommon in Asian countries due to low population frequency of celiac HLA risk alleles<ref name=":6" /><ref name=":1" /><ref name=":2" />


==Clinical Features==
==Clinical Features==
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*Weight loss
*Weight loss
*Gluten-insensitive diarrhea/malabsorption
*Gluten-insensitive diarrhea/malabsorption
*Bowel obstruction or perforation
*Bowel obstruction or perforation (50% cases)
|-
|-
|'''Laboratory Findings'''
|'''Laboratory Findings'''
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*Hemophagocytosis
*Hemophagocytosis
|}
|}
* '''CD can be diagnosed at the time of EATL diagnosis'''<ref name=":5" />
* '''Patients with RCD can sometimes present with small-intestinal ulceration (ulcerative jejunitis) { 9250161 ; 10381521 }.'''


If there is no prior diagnosis of celiac disease and lymphoma is the initial presentation, the following findings can point towards celiac disease associated EATL:
If there is no prior diagnosis of celiac disease and lymphoma is the initial presentation, the following findings can point towards celiac disease associated EATL:
* Anti-tissue transglutaminase-2 antibodies or Anti-endomysial antibodies
* Dermatitis herpetiformis
<blockquote class="blockedit">{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}
Many of the below features are indistinguishable from the presentation of celiac disease, which may delay the diagnosis of EATL. Persistent symptoms following gluten-free diet is highly suggestive of EATL.<ref name=":1" />
'''Signs & Symptoms'''
*Abdominal pain
*Weight loss
*Gluten-insensitive diarrhea/malabsorption
*Bowel obstruction or perforation
'''Laboratory Findings'''
*Anemia
*Hypoalbuminemia
*Hemophagocytosis
If there is no prior diagnosis of celiac disease and lymphoma is the initial presentation, the following findings can point towards celiac disease associated EATL:


*Anti-tissue transglutaminase-2 antibodies or Anti-endomysial antibodies
*Anti-tissue transglutaminase-2 antibodies or Anti-endomysial antibodies
*Dermatitis herpetiformis
*Dermatitis herpetiformis


</blockquote>
==Sites of Involvement==
==Sites of Involvement==


*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" />'''
*Metastasis involve intra-abdominal node > bone marrow > lung > liver > skin<ref name=":1" />
*'''Dissemination to extra gastrointestinal sites: mesenteric and abdominal lymph nodes > bone marrow, lung, liver or skin { 8583077 ; 23313469 ; 21566094 }.'''
*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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|}
|}


Immunophenotype of intraepithelial lymphocytes (IEL):<ref name=":7" /><ref name=":0" />
{| class="wikitable sortable"
|-
!Finding
!Type 1 RCD!!Type 2 RCD
|-
|Positive (universal)
| ||CD3, CD7
|-
|Positive (frequent)
| ||CD30 (harbinger of transformation to EATL from RCD2), NKP46 (not seen in IEL of CD or RCD1), CD103,
cytotoxic granule-associated markers (TIA1, granzyme B, perforin)
|-
|Negative (frequent)
| ||CD4, CD8, CD5, CD56, TCR, EBER
|-
|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>


*Varies depending on background type 1 or type 2 refractory celiac disease (RCD).
*Varies depending on background type 1 or type 2 refractory celiac disease (RCD).
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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>'''


<blockquote class="blockedit">{{Box-round|title=v4:Immunophenotype|The content below was from the old template. Please incorporate above.}}
{| class="wikitable sortable"
|-
!Finding<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>!!Marker
|-
|Positive (universal)||CD3, CD7
|-
|Positive (frequent)||CD30 (harbinger of transformation to EATL from RCD2), NKP46 (not seen in IEL of CD or RCD1), CD103,
cytotoxic granule-associated markers (TIA1, granzyme B, perforin)
|-
|Negative (frequent)
|CD4, CD8, CD5, CD56, TCR
|-
|Ki-67
|high
|}
*Immunophenotype of intraepithelial lymphocytes (IEL):<ref name=":7" /><ref name=":0" />
**Varies depending on background type 1 or type 2 refractory celiac disease (RCD).
***Type 1 (RCD1):
****Milder symptoms with high 5-year survival with low risk of EATL development
****Flow cytometry: sCD3+, CD8+, CD5+
***Type 2 (RCD2):
****Severe symptoms with protein-losing enteropathy leads to malnourishment (BMI < 18); low 5-year survival with increased risk of EATL
****Flow cytometry: sCD3<sup>_</sup>, CD8-, CD5-
****IHC:
*****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
****
</blockquote>
==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==


No recurrent gene fusions have been reported.
No recurrent gene fusions have been reported.<blockquote class="blockedit">{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
 
{| class="wikitable sortable"
|-
!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 20% (COSMIC)
EXAMPLE 30% (add reference)
|Yes
|No
|Yes
|EXAMPLE
 
The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).
|}
 
<blockquote class="blockedit">{{Box-round|title=v4:Chromosomal Rearrangements (Gene Fusions)|The content below was from the old template. Please incorporate above.}}
 
*No recurrent gene fusions reported
 
</blockquote>
 
 
<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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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
!Gene; Genetic Alteration!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC /  TCGA / Other)'''!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''
!Gene; Genetic Alteration<ref name=":4" /><ref>{{Cite journal|last=Sh|first=Swerdlow|last2=Jr|first2=Cook|date=2020|title=As the world turns, evolving lymphoma classifications-past, present and future|url=https://pubmed.ncbi.nlm.nih.gov/31493426/|language=en|pmid=31493426}}</ref>!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC /  TCGA / Other)'''<ref name=":4" />!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''
!'''Diagnostic Significance (Yes, No or Unknown)'''
!'''Diagnostic Significance (Yes, No or Unknown)'''
!Prognostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
Line 438: Line 380:
!Notes
!Notes
|-
|-
|EXAMPLE: TP53; Variable LOF mutations
|SETD2
 
|TSG
EXAMPLE:
|32%
 
|
EGFR; Exon 20 mutations
|
 
|
EXAMPLE: BRAF; Activating mutations
|EXAMPLE: TSG
|EXAMPLE: 20% (COSMIC)
 
EXAMPLE: 30% (add Reference)
|EXAMPLE: IDH1 R123H
|EXAMPLE: EGFR amplification
|
|
|
|
|
|
|EXAMPLE:  Excludes hairy cell leukemia (HCL) (add reference).
<br />
|}
Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) 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.}}
{| class="wikitable"
!Gene*<ref name=":4" /><ref>{{Cite journal|last=Sh|first=Swerdlow|last2=Jr|first2=Cook|date=2020|title=As the world turns, evolving lymphoma classifications-past, present and future|url=https://pubmed.ncbi.nlm.nih.gov/31493426/|language=en|pmid=31493426}}</ref>
!Function/Oncogene/Tumor Suppressor Gene
!Frequency<ref name=":4" />
|-
|SETD2
|Tumor suppressor gene
|32%
|-
|-
|YLPM1
|YLPM1
|Tumor suppressor gene
|TSG
|22%
|22%
|
|
|
|
|
|
|-
|-
|TET2
|TET2
|Tumor suppressor gene
|TSG
|14%
|14%
|
|
|
|
|
|
|-
|-
|STAT5B
|STAT5B
|Oncogene
|Oncogene
|29%
|29%
|
|
|
|
|
|
|-
|-
|JAK1
|JAK1
|Oncogene
|Oncogene
|23%
|23%
|
|
|
|
|
|
|-
|-
|JAK3
|JAK3
|Oncogene
|Oncogene
|23%
|23%
|
|
|
|
|
|
|-
|-
|STAT3
|STAT3
|Oncogene
|oncogene
|16%
|16%
|
|
|
|
|
|
|-
|-
|SOCS1
|SOCS1
|Tumor suppressor gene
|TSG
|7%
|7%
|
|
|
|
|
|
|-
|-
|NRAS
|NRAS
|Oncogene
|Oncogene
|10%
|10%
|
|
|
|
|
|
|-
|-
|KRAS
|KRAS
|Oncogene
|Oncogene
|6%
|6%
|
|
|
|
|
|
|-
|-
|TP53
|TP53
|Tumor suppressor gene
|TSG
|10%
|10%
|
|
|
|
|
|
|-
|-
|BCL11B
|BCL11B
|Tumor suppressor gene
|TSG
|13%
|13%
|
|
|
|
|
|
|-
|-
|BRIP1
|BRIP1
|Tumor suppressor gene
|TSG
|16%
|16%
|
|
|
|
|
|
|-
|-
|TERT
|TERT
|Oncogene
|Oncogene
|17%
|17%
|
|
|
|
|
|
|-
|-
|BBX
|BBX
|Cell cycle transcription factor
|Cell cycle transcription factor
|16%
|16%
|
|
|
|
|
|
|-
|-
|DAPK3
|DAPK3
|Apoptosis
|Apoptosis
|10%
|10%
|
|
|
|
|
|
|-
|-
|PRDM1
|PRDM1
|Interferon-related transcription factor
|Interferon-related transcription factor
|9%
|9%
|
|
|
|
|
|
|}
|}
Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
<nowiki>*</nowiki>The specific mutations in these genes may be found elsewhere ([https://cancer.sanger.ac.uk/cosmic COSMIC], [https://www.cbioportal.org/ cBioPortal])
<nowiki>*</nowiki>The specific mutations in these genes may be found elsewhere ([https://cancer.sanger.ac.uk/cosmic COSMIC], [https://www.cbioportal.org/ cBioPortal])


*''PRDM1'' and ''DAPK3'', followed by ''STAT3'' and ''STAT5B'', are the most common mutually exclusive gene pairs<ref name=":4" />
*''PRDM1'' and ''DAPK3'', followed by ''STAT3'' and ''STAT5B'', are the most common mutually exclusive gene pairs<ref name=":4" />


</blockquote>
==Epigenomic Alterations==
==Epigenomic Alterations==


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