HAEM5:Large B-cell lymphoma with IRF4 rearrangement: Difference between revisions

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{{DISPLAYTITLE:Large B-cell lymphoma with IRF4 rearrangement}}
{{DISPLAYTITLE:Large B-cell lymphoma with IRF4 rearrangement}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]


{{Under Construction}}
{{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:Large B-cell Lymphoma with IRF4 Rearrangement]].
<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:Large B-cell Lymphoma with IRF4 Rearrangement]].
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==Definition / Description of Disease==
*Rare LBCL harboring ''IRF4'' gene rearrangement and involving head & neck lymphoid tissues typically in the pediatric age group<ref>{{Cite journal|last=Liu|first=Qingyan|last2=Salaverria|first2=Itziar|last3=Pittaluga|first3=Stefania|last4=Jegalian|first4=Armin G.|last5=Xi|first5=Liqiang|last6=Siebert|first6=Reiner|last7=Raffeld|first7=Mark|last8=Hewitt|first8=Stephen M.|last9=Jaffe|first9=Elaine S.|date=2013-03|title=Follicular lymphomas in children and young adults: a comparison of the pediatric variant with usual follicular lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/23108024|journal=The American Journal of Surgical Pathology|volume=37|issue=3|pages=333–343|doi=10.1097/PAS.0b013e31826b9b57|issn=1532-0979|pmc=3566339|pmid=23108024}}</ref><ref>{{Cite journal|last=Louissaint|first=Abner|last2=Ackerman|first2=Adam M.|last3=Dias-Santagata|first3=Dora|last4=Ferry|first4=Judith A.|last5=Hochberg|first5=Ephraim P.|last6=Huang|first6=Mary S.|last7=Iafrate|first7=A. John|last8=Lara|first8=Daniel O.|last9=Pinkus|first9=Geraldine S.|date=2012-09-20|title=Pediatric-type nodal follicular lymphoma: an indolent clonal proliferation in children and adults with high proliferation index and no BCL2 rearrangement|url=https://pubmed.ncbi.nlm.nih.gov/22855608|journal=Blood|volume=120|issue=12|pages=2395–2404|doi=10.1182/blood-2012-05-429514|issn=1528-0020|pmid=22855608}}</ref><ref name=":0">{{Cite journal|last=Salaverria|first=Itziar|last2=Philipp|first2=Claudia|last3=Oschlies|first3=Ilske|last4=Kohler|first4=Christian W.|last5=Kreuz|first5=Markus|last6=Szczepanowski|first6=Monika|last7=Burkhardt|first7=Birgit|last8=Trautmann|first8=Heiko|last9=Gesk|first9=Stefan|date=2011-07-07|title=Translocations activating IRF4 identify a subtype of germinal center-derived B-cell lymphoma affecting predominantly children and young adults|url=https://ashpublications.org/blood/article/118/1/139/28404/Translocations-activating-IRF4-identify-a-subtype|journal=Blood|language=en|volume=118|issue=1|pages=139–147|doi=10.1182/blood-2011-01-330795|issn=0006-4971}}</ref>
<references group="23108024" />
==Synonyms / Terminology==
*None
==Epidemiology / Prevalence==
*Rare ~0.05% of LBCLs
*Median age 12 years
*Male ~ Female
<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=":1">{{Cite journal|last=de Leval|first=L.|last2=Bonnet|first2=C.|last3=Copie-Bergman|first3=C.|last4=Seidel|first4=L.|last5=Baia|first5=M.|last6=Brière|first6=J.|last7=Molina|first7=T. J.|last8=Fabiani|first8=B.|last9=Petrella|first9=T.|date=2012-12|title=Diffuse large B-cell lymphoma of Waldeyer's ring has distinct clinicopathologic features: a GELA study|url=https://pubmed.ncbi.nlm.nih.gov/22700993|journal=Annals of Oncology: Official Journal of the European Society for Medical Oncology|volume=23|issue=12|pages=3143–3151|doi=10.1093/annonc/mds150|issn=1569-8041|pmid=22700993}}</ref><ref name=":2">{{Cite journal|last=Chisholm|first=Karen M.|last2=Mohlman|first2=Jeffrey|last3=Liew|first3=Michael|last4=Termuhlen|first4=Amanda|last5=Cairo|first5=Mitchell S.|last6=Gross|first6=Thomas G.|last7=Perkins|first7=Sherrie L.|last8=Miles|first8=Rodney R.|date=2019-08|title=IRF4 translocation status in pediatric follicular and diffuse large B‐cell lymphoma patients enrolled in Children's Oncology Group trials|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/pbc.27770|journal=Pediatric Blood & Cancer|language=en|volume=66|issue=8|doi=10.1002/pbc.27770|issn=1545-5009}}</ref><blockquote class="blockedit">
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==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)
|}
<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>
*Lymphadenopathy
*Tonsillar hypertrophy
<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=":1" /><ref name=":2" /><blockquote class="blockedit">
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==Sites of Involvement==
*Head and neck lymph nodes
*Waldeyer ring
*Gastrointestinal tract
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==Morphologic Features==
*Medium to large sized neoplastic cells
*Chromatin is more open than seen in centrocytes
*Small basophilic nucleoli
*Follicular type: large neoplastic follicles are seen with a back-to-back growth pattern and absent or attenuated mantle zones
*Follicles lack serpiginous configuration and starry-sky pattern
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==Immunophenotype==
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||CD20, CD79a, PAX5, BCL6, MUM1
|-
|Positive (subset)||CD10, BCL2
|-
|Negative (universal)||PRDM1
|}
<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=":2" /><ref>{{Cite journal|last=Sukswai|first=Narittee|last2=Lyapichev|first2=Kirill|last3=Khoury|first3=Joseph D.|last4=Medeiros|first4=L. Jeffrey|date=2020-01|title=Diffuse large B-cell lymphoma variants: an update|url=https://pubmed.ncbi.nlm.nih.gov/31735345|journal=Pathology|volume=52|issue=1|pages=53–67|doi=10.1016/j.pathol.2019.08.013|issn=1465-3931|pmid=31735345}}</ref><blockquote class="blockedit">
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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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<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=Ramis-Zaldivar|first=Joan Enric|last2=Gonzalez-Farré|first2=Blanca|last3=Balagué|first3=Olga|last4=Celis|first4=Verónica|last5=Nadeu|first5=Ferran|last6=Salmerón-Villalobos|first6=Julia|last7=Andrés|first7=Mara|last8=Martin-Guerrero|first8=Idoia|last9=Garrido-Pontnou|first9=Marta|date=01 23, 2020|title=Distinct molecular profile of IRF4-rearranged large B-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/31738823|journal=Blood|volume=135|issue=4|pages=274–286|doi=10.1182/blood.2019002699|issn=1528-0020|pmc=6978155|pmid=31738823}}</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=Salaverria|first=Itziar|last2=Philipp|first2=Claudia|last3=Oschlies|first3=Ilske|last4=Kohler|first4=Christian W.|last5=Kreuz|first5=Markus|last6=Szczepanowski|first6=Monika|last7=Burkhardt|first7=Birgit|last8=Trautmann|first8=Heiko|last9=Gesk|first9=Stefan|date=2011-07-07|title=Translocations activating IRF4 identify a subtype of germinal center-derived B-cell lymphoma affecting predominantly children and young adults|url=https://ashpublications.org/blood/article/118/1/139/28404/Translocations-activating-IRF4-identify-a-subtype|journal=Blood|language=en|volume=118|issue=1|pages=139–147|doi=10.1182/blood-2011-01-330795|issn=0006-4971}}</ref><ref>{{Cite journal|last=Ramis-Zaldivar|first=Joan Enric|last2=Gonzalez-Farré|first2=Blanca|last3=Balagué|first3=Olga|last4=Celis|first4=Verónica|last5=Nadeu|first5=Ferran|last6=Salmerón-Villalobos|first6=Julia|last7=Andrés|first7=Mara|last8=Martin-Guerrero|first8=Idoia|last9=Garrido-Pontnou|first9=Marta|date=01 23, 2020|title=Distinct molecular profile of IRF4-rearranged large B-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/31738823|journal=Blood|volume=135|issue=4|pages=274–286|doi=10.1182/blood.2019002699|issn=1528-0020|pmc=6978155|pmid=31738823}}</ref><blockquote class="blockedit">
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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 name=":0" /><ref name=":2" /><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 name=":2">{{Cite journal|last=Chisholm|first=Karen M.|last2=Mohlman|first2=Jeffrey|last3=Liew|first3=Michael|last4=Termuhlen|first4=Amanda|last5=Cairo|first5=Mitchell S.|last6=Gross|first6=Thomas G.|last7=Perkins|first7=Sherrie L.|last8=Miles|first8=Rodney R.|date=2019-08|title=IRF4 translocation status in pediatric follicular and diffuse large B‐cell lymphoma patients enrolled in Children's Oncology Group trials|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/pbc.27770|journal=Pediatric Blood & Cancer|language=en|volume=66|issue=8|doi=10.1002/pbc.27770|issn=1545-5009}}</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>{{Cite journal|last=Salaverria|first=Itziar|last2=Martin-Guerrero|first2=Idoia|last3=Burkhardt|first3=Birgit|last4=Kreuz|first4=Markus|last5=Zenz|first5=Thorsten|last6=Oschlies|first6=Ilske|last7=Arnold|first7=Norbert|last8=Baudis|first8=Michael|last9=Bens|first9=Susanne|date=2013-02|title=High resolution copy number analysis of IRF4 translocation-positive diffuse large B-cell and follicular lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/23073988|journal=Genes, Chromosomes & Cancer|volume=52|issue=2|pages=150–155|doi=10.1002/gcc.22014|issn=1098-2264|pmid=23073988}}</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=Salaverria|first=Itziar|last2=Martin-Guerrero|first2=Idoia|last3=Burkhardt|first3=Birgit|last4=Kreuz|first4=Markus|last5=Zenz|first5=Thorsten|last6=Oschlies|first6=Ilske|last7=Arnold|first7=Norbert|last8=Baudis|first8=Michael|last9=Bens|first9=Susanne|date=2013-02|title=High resolution copy number analysis of IRF4 translocation-positive diffuse large B-cell and follicular lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/23073988|journal=Genes, Chromosomes & Cancer|volume=52|issue=2|pages=150–155|doi=10.1002/gcc.22014|issn=1098-2264|pmid=23073988}}</ref><blockquote class="blockedit">
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''IRF4'' Rearrangement<ref name=":0" />
''IRF4'' Rearrangement<ref name=":0" />
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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.


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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=":3">{{Cite journal|last=Ramis-Zaldivar|first=Joan Enric|last2=Gonzalez-Farré|first2=Blanca|last3=Balagué|first3=Olga|last4=Celis|first4=Verónica|last5=Nadeu|first5=Ferran|last6=Salmerón-Villalobos|first6=Julia|last7=Andrés|first7=Mara|last8=Martin-Guerrero|first8=Idoia|last9=Garrido-Pontnou|first9=Marta|date=01 23, 2020|title=Distinct molecular profile of IRF4-rearranged large B-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/31738823|journal=Blood|volume=135|issue=4|pages=274–286|doi=10.1182/blood.2019002699|issn=1528-0020|pmc=6978155|pmid=31738823}}</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=":3">{{Cite journal|last=Ramis-Zaldivar|first=Joan Enric|last2=Gonzalez-Farré|first2=Blanca|last3=Balagué|first3=Olga|last4=Celis|first4=Verónica|last5=Nadeu|first5=Ferran|last6=Salmerón-Villalobos|first6=Julia|last7=Andrés|first7=Mara|last8=Martin-Guerrero|first8=Idoia|last9=Garrido-Pontnou|first9=Marta|date=01 23, 2020|title=Distinct molecular profile of IRF4-rearranged large B-cell lymphoma|url=https://pubmed.ncbi.nlm.nih.gov/31738823|journal=Blood|volume=135|issue=4|pages=274–286|doi=10.1182/blood.2019002699|issn=1528-0020|pmc=6978155|pmid=31738823}}</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>{{Cite journal|last=Salaverria|first=Itziar|last2=Martin-Guerrero|first2=Idoia|last3=Burkhardt|first3=Birgit|last4=Kreuz|first4=Markus|last5=Zenz|first5=Thorsten|last6=Oschlies|first6=Ilske|last7=Arnold|first7=Norbert|last8=Baudis|first8=Michael|last9=Bens|first9=Susanne|date=2013-02|title=High resolution copy number analysis of IRF4 translocation-positive diffuse large B-cell and follicular lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/23073988|journal=Genes, Chromosomes & Cancer|volume=52|issue=2|pages=150–155|doi=10.1002/gcc.22014|issn=1098-2264|pmid=23073988}}</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=Salaverria|first=Itziar|last2=Martin-Guerrero|first2=Idoia|last3=Burkhardt|first3=Birgit|last4=Kreuz|first4=Markus|last5=Zenz|first5=Thorsten|last6=Oschlies|first6=Ilske|last7=Arnold|first7=Norbert|last8=Baudis|first8=Michael|last9=Bens|first9=Susanne|date=2013-02|title=High resolution copy number analysis of IRF4 translocation-positive diffuse large B-cell and follicular lymphomas|url=https://pubmed.ncbi.nlm.nih.gov/23073988|journal=Genes, Chromosomes & Cancer|volume=52|issue=2|pages=150–155|doi=10.1002/gcc.22014|issn=1098-2264|pmid=23073988}}</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 />
(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'': “Large B-cell lymphoma with IRF4 rearrangement”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Large_B-cell_lymphoma_with_IRF4_rearrangement</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Large B-cell lymphoma with IRF4 rearrangement”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Large_B-cell_lymphoma_with_IRF4_rearrangement</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases L]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases L]]