HAEM5:Paediatric nodal marginal zone lymphoma - Revision history

Bailey.Glen at 17:25, 3 July 2025

2025-07-03T17:25:32Z

← Older revision		Revision as of 12:25, 3 July 2025
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	\|}		\|}

	~~==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>
	~~{\| class="wikitable"~~
	\|+
	\|WHO Essential Criteria (Genetics)*
	\|
	\|-
	\|WHO Desirable Criteria (Genetics)*
	\|
	\|-
	~~\|Other Classification~~
	\|
	\|}
	<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
	==Related Terminology==		==Related Terminology==

Bailey.Glen at 21:45, 25 May 2025

2025-05-25T21:45:48Z

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	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Chr #!!~~'''~~Gain, Loss, Amp, LOH~~'''~~!!~~'''~~Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]~~'''~~!!~~'''~~Relevant Gene(s)~~'''~~		!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>		\|<span class="blue-text">EXAMPLE:</span>
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	!Chromosomal Pattern		!Chromosomal Pattern
	!Molecular Pathogenesis		!Molecular Pathogenesis
	!~~'''~~Prevalence -~~'''~~		!Prevalence -
	~~'''~~Common >20%, Recurrent 5-20% or Rare <5% (Disease)~~'''~~		Common >20%, Recurrent 5-20% or Rare <5% (Disease)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>		\|<span class="blue-text">EXAMPLE:</span>
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	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Gene!!~~'''~~Genetic Alteration~~'''~~!!~~'''~~Tumor Suppressor Gene, Oncogene, Other~~'''~~!!~~'''~~Prevalence -~~'''~~		!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
	~~'''~~Common >20%, Recurrent 5-20% or Rare <5% (Disease)~~'''~~		Common >20%, Recurrent 5-20% or Rare <5% (Disease)
	!~~'''~~Diagnostic, Prognostic, and Therapeutic Significance - D, P, T ~~'''~~		!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
	!~~'''~~Established Clinical Significance Per Guidelines - Yes or No (Source)~~'''~~		!Established Clinical Significance Per Guidelines - Yes or No (Source)
	!~~'''~~Clinical Relevance Details/Other Notes~~'''~~		!Clinical Relevance Details/Other Notes
	\|-		\|-
	\|<span class="blue-text">EXAMPLE:</span>''EGFR''		\|<span class="blue-text">EXAMPLE:</span>''EGFR''

Bailey.Glen at 20:21, 25 May 2025

2025-05-25T20:21:19Z

← Older revision		Revision as of 15:21, 25 May 2025
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	<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].		<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
	==Related Terminology==		==Related Terminology==
	~~<span style="color:#0070C0">(''Instructions: The table will have the related terminology from the WHO <u>autocompleted</u>.)''</span>~~
	{\| class="wikitable"		{\| class="wikitable"
	\|+		\|+
	\|Acceptable		\|Acceptable
	\|		\|N/A
	\|-		\|-
	\|Not Recommended		\|Not Recommended
	\|		\|Monocytoid B-cell lymphoma; parafollicular B-cell lymphoma
	\|}		\|}

Bailey.Glen at 17:43, 24 March 2025

2025-03-24T17:43:49Z

← Older revision		Revision as of 12:43, 24 March 2025
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	{{DISPLAYTITLE:Paediatric nodal marginal zone lymphoma}}		{{DISPLAYTITLE:Paediatric nodal marginal zone 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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	*Kathleen M. Schieffer, PhD		*Kathleen M. Schieffer, PhD
	*Ruthann Pfau, PhD		*Ruthann Pfau, PhD

	~~__TOC__~~

	==WHO Classification of Disease==		==WHO Classification of Disease==

Bailey.Glen: Removed old template contents

2025-02-11T17:48:41Z

Removed old template contents

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Bailey.Glen at 18:57, 10 February 2025

2025-02-10T18:57:18Z

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Bailey.Glen at 18:29, 10 February 2025

2025-02-10T18:29:17Z

← Older revision		Revision as of 13:29, 10 February 2025
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	<blockquote class='blockedit'>{{Box-round\|title=v4:Clinical Features\|The content below was from the old template. Please incorporate above.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Clinical Features\|The content below was from the old template. Please incorporate above.}}</blockquote>

	* Asymptomatic in most<ref name=":6" /><ref name=":8" /><ref name=":11" /><ref name=":12" />		* Asymptomatic in most<ref name=":6" /><ref name=":8" /><ref name=":11" /><ref name=":12" />
	* Peripheral lymphadenopathy<ref name=":6" /><ref name=":8" /><ref name=":11" /><ref name=":12">{{Cite journal\|last=Attarbaschi\|first=Andishe\|last2=Abla\|first2=Oussama\|last3=Arias Padilla\|first3=Laura\|last4=Beishuizen\|first4=Auke\|last5=Burke\|first5=G. A. Amos\|last6=Brugières\|first6=Laurence\|last7=Bruneau\|first7=Julie\|last8=Burkhardt\|first8=Birgit\|last9=d'Amore\|first9=Emanuele S. G.\|date=08 2020\|title=Rare non-Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric-type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T-cell lymphoma\|url=https://pubmed.ncbi.nlm.nih.gov/32452165\|journal=Pediatric Blood & Cancer\|volume=67\|issue=8\|pages=e28416\|doi=10.1002/pbc.28416\|issn=1545-5017\|pmid=32452165}}</ref>		* Peripheral lymphadenopathy<ref name=":6" /><ref name=":8" /><ref name=":11" /><ref name=":12">{{Cite journal\|last=Attarbaschi\|first=Andishe\|last2=Abla\|first2=Oussama\|last3=Arias Padilla\|first3=Laura\|last4=Beishuizen\|first4=Auke\|last5=Burke\|first5=G. A. Amos\|last6=Brugières\|first6=Laurence\|last7=Bruneau\|first7=Julie\|last8=Burkhardt\|first8=Birgit\|last9=d'Amore\|first9=Emanuele S. G.\|date=08 2020\|title=Rare non-Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric-type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T-cell lymphoma\|url=https://pubmed.ncbi.nlm.nih.gov/32452165\|journal=Pediatric Blood & Cancer\|volume=67\|issue=8\|pages=e28416\|doi=10.1002/pbc.28416\|issn=1545-5017\|pmid=32452165}}</ref>

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==Sites of Involvement==		==Sites of Involvement==
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	<blockquote class='blockedit'>{{Box-round\|title=v4:Chromosomal Rearrangements (Gene Fusions)\|The content below was from the old template. Please incorporate above.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Chromosomal Rearrangements (Gene Fusions)\|The content below was from the old template. Please incorporate above.}}</blockquote>

	* No chromosomal rearrangements or gene fusions associated with pNMZL<ref name=":0" />		* No chromosomal rearrangements or gene fusions associated with pNMZL<ref name=":0" />
	* Clonal rearrangements of immunoglobulin (Ig) region detected in most cases<ref name=":3" /><ref name=":9" />		* Clonal rearrangements of immunoglobulin (Ig) region detected in most cases<ref name=":3" /><ref name=":9" />

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>

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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>

	* No genomic findings currently assist in diagnosis, prognostication, or therapeutic decisions		* No genomic findings currently assist in diagnosis, prognostication, or therapeutic decisions

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==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.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Genomic Gain/Loss/LOH\|The content below was from the old template. Please incorporate above.}}</blockquote>

	* Trisomy 3 and 18 are infrequently described chromosomal aberrations reported in pNMZL<ref name=":9" />		* Trisomy 3 and 18 are infrequently described chromosomal aberrations reported in pNMZL<ref name=":9" />
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	\|}		\|}

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==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 characteristic chromosomal aberrations or patterns reported		* No characteristic chromosomal aberrations or patterns reported

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==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.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Gene Mutations (SNV/INDEL)\|The content below was from the old template. Please incorporate above.}}</blockquote>

	* Genes reported to be altered in adult-type NMZL, including ''MLL2 (KMT2D), PTPRD, NOTCH2, KLF2,'' and ''BRAF'',<ref>{{Cite journal\|last=Spina\|first=Valeria\|last2=Khiabanian\|first2=Hossein\|last3=Messina\|first3=Monica\|last4=Monti\|first4=Sara\|last5=Cascione\|first5=Luciano\|last6=Bruscaggin\|first6=Alessio\|last7=Spaccarotella\|first7=Elisa\|last8=Holmes\|first8=Antony B.\|last9=Arcaini\|first9=Luca\|date=09 08, 2016\|title=The genetics of nodal marginal zone lymphoma\|url=https://pubmed.ncbi.nlm.nih.gov/27335277\|journal=Blood\|volume=128\|issue=10\|pages=1362–1373\|doi=10.1182/blood-2016-02-696757\|issn=1528-0020\|pmc=5016706\|pmid=27335277}}</ref><ref>{{Cite journal\|last=Pillonel\|first=V.\|last2=Juskevicius\|first2=D.\|last3=Ng\|first3=C. K. Y.\|last4=Bodmer\|first4=A.\|last5=Zettl\|first5=A.\|last6=Jucker\|first6=D.\|last7=Dirnhofer\|first7=S.\|last8=Tzankov\|first8=A.\|date=11 2018\|title=High-throughput sequencing of nodal marginal zone lymphomas identifies recurrent BRAF mutations\|url=https://pubmed.ncbi.nlm.nih.gov/29556019\|journal=Leukemia\|volume=32\|issue=11\|pages=2412–2426\|doi=10.1038/s41375-018-0082-4\|issn=1476-5551\|pmc=6224405\|pmid=29556019}}</ref> have not been described in pNMZL<ref name=":0" />		* Genes reported to be altered in adult-type NMZL, including ''MLL2 (KMT2D), PTPRD, NOTCH2, KLF2,'' and ''BRAF'',<ref>{{Cite journal\|last=Spina\|first=Valeria\|last2=Khiabanian\|first2=Hossein\|last3=Messina\|first3=Monica\|last4=Monti\|first4=Sara\|last5=Cascione\|first5=Luciano\|last6=Bruscaggin\|first6=Alessio\|last7=Spaccarotella\|first7=Elisa\|last8=Holmes\|first8=Antony B.\|last9=Arcaini\|first9=Luca\|date=09 08, 2016\|title=The genetics of nodal marginal zone lymphoma\|url=https://pubmed.ncbi.nlm.nih.gov/27335277\|journal=Blood\|volume=128\|issue=10\|pages=1362–1373\|doi=10.1182/blood-2016-02-696757\|issn=1528-0020\|pmc=5016706\|pmid=27335277}}</ref><ref>{{Cite journal\|last=Pillonel\|first=V.\|last2=Juskevicius\|first2=D.\|last3=Ng\|first3=C. K. Y.\|last4=Bodmer\|first4=A.\|last5=Zettl\|first5=A.\|last6=Jucker\|first6=D.\|last7=Dirnhofer\|first7=S.\|last8=Tzankov\|first8=A.\|date=11 2018\|title=High-throughput sequencing of nodal marginal zone lymphomas identifies recurrent BRAF mutations\|url=https://pubmed.ncbi.nlm.nih.gov/29556019\|journal=Leukemia\|volume=32\|issue=11\|pages=2412–2426\|doi=10.1038/s41375-018-0082-4\|issn=1476-5551\|pmc=6224405\|pmid=29556019}}</ref> have not been described in pNMZL<ref name=":0" />
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	* Additional studies are needed to assess the spectrum of somatic variation in pNMZL. A single report evaluated the genetic landscape of pNMZL by whole exome sequencing (n=4) identified missense changes in the following genes: ''AMOTL1, SCAF1, SELPLG, FAM5B, KLHDC4, RAX, PEG3, CHPF, ACTRT3, NRCAM, CHMP1A, CISH, TTC17, NLE1<ref name=":0" />''		* Additional studies are needed to assess the spectrum of somatic variation in pNMZL. A single report evaluated the genetic landscape of pNMZL by whole exome sequencing (n=4) identified missense changes in the following genes: ''AMOTL1, SCAF1, SELPLG, FAM5B, KLHDC4, RAX, PEG3, CHPF, ACTRT3, NRCAM, CHMP1A, CISH, TTC17, NLE1<ref name=":0" />''

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==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.}}		<blockquote class='blockedit'>{{Box-round\|title=v4:Genes and Main Pathways Involved\|The content below was from the old template. Please incorporate above.}}</blockquote>

	* ''AMOTL1'' encodes angiomotin like-1 which associates with tight junctions and regulates the Hippo signaling pathway<ref>{{Cite journal\|last=Yi\|first=Chunling\|last2=Troutman\|first2=Scott\|last3=Fera\|first3=Daniela\|last4=Stemmer-Rachamimov\|first4=Anat\|last5=Avila\|first5=Jacqueline L.\|last6=Christian\|first6=Neepa\|last7=Persson\|first7=Nathalie Luna\|last8=Shimono\|first8=Akihiko\|last9=Speicher\|first9=David W.\|date=2011-04-12\|title=A tight junction-associated Merlin-angiomotin complex mediates Merlin's regulation of mitogenic signaling and tumor suppressive functions\|url=https://pubmed.ncbi.nlm.nih.gov/21481793\|journal=Cancer Cell\|volume=19\|issue=4\|pages=527–540\|doi=10.1016/j.ccr.2011.02.017\|issn=1878-3686\|pmc=3075552\|pmid=21481793}}</ref><ref>{{Cite journal\|last=Lv\|first=Meng\|last2=Shen\|first2=Yanwei\|last3=Yang\|first3=Jiao\|last4=Li\|first4=Shuting\|last5=Wang\|first5=Biyuan\|last6=Chen\|first6=Zheling\|last7=Li\|first7=Pan\|last8=Liu\|first8=Peijun\|last9=Yang\|first9=Jin\|date=2017\|title=Angiomotin Family Members: Oncogenes or Tumor Suppressors?\|url=https://pubmed.ncbi.nlm.nih.gov/28656002\|journal=International Journal of Biological Sciences\|volume=13\|issue=6\|pages=772–781\|doi=10.7150/ijbs.19603\|issn=1449-2288\|pmc=5485632\|pmid=28656002}}</ref>		* ''AMOTL1'' encodes angiomotin like-1 which associates with tight junctions and regulates the Hippo signaling pathway<ref>{{Cite journal\|last=Yi\|first=Chunling\|last2=Troutman\|first2=Scott\|last3=Fera\|first3=Daniela\|last4=Stemmer-Rachamimov\|first4=Anat\|last5=Avila\|first5=Jacqueline L.\|last6=Christian\|first6=Neepa\|last7=Persson\|first7=Nathalie Luna\|last8=Shimono\|first8=Akihiko\|last9=Speicher\|first9=David W.\|date=2011-04-12\|title=A tight junction-associated Merlin-angiomotin complex mediates Merlin's regulation of mitogenic signaling and tumor suppressive functions\|url=https://pubmed.ncbi.nlm.nih.gov/21481793\|journal=Cancer Cell\|volume=19\|issue=4\|pages=527–540\|doi=10.1016/j.ccr.2011.02.017\|issn=1878-3686\|pmc=3075552\|pmid=21481793}}</ref><ref>{{Cite journal\|last=Lv\|first=Meng\|last2=Shen\|first2=Yanwei\|last3=Yang\|first3=Jiao\|last4=Li\|first4=Shuting\|last5=Wang\|first5=Biyuan\|last6=Chen\|first6=Zheling\|last7=Li\|first7=Pan\|last8=Liu\|first8=Peijun\|last9=Yang\|first9=Jin\|date=2017\|title=Angiomotin Family Members: Oncogenes or Tumor Suppressors?\|url=https://pubmed.ncbi.nlm.nih.gov/28656002\|journal=International Journal of Biological Sciences\|volume=13\|issue=6\|pages=772–781\|doi=10.7150/ijbs.19603\|issn=1449-2288\|pmc=5485632\|pmid=28656002}}</ref>

			<blockquote class="blockedit">
			<center><span style="color:Maroon">'''End of V4 Section'''</span>
			----
	</blockquote>		</blockquote>
	==Genetic Diagnostic Testing Methods==		==Genetic Diagnostic Testing Methods==

Bailey.Glen at 22:28, 6 September 2024

2024-09-06T22:28:33Z

← Older revision		Revision as of 17:28, 6 September 2024
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	__TOC__		__TOC__

	==~~Cancer Category / Type~~==		==WHO Classification of Disease==

	* [[HAEM4:Mature B-~~Cell Neoplasms]]~~		{\| class="wikitable"
			!Structure
	~~==Cancer Sub~~-~~Classification /~~ Subtype==		!Disease
			\|-
	* [[HAEM5:Nodal marginal zone lymphoma~~]] (NMZL)~~		\|Book
			\|Haematolymphoid Tumours (5th ed.)
			\|-
			\|Category
			\|B-cell lymphoid proliferations and lymphomas
			\|-
			\|Family
			\|Mature B-cell neoplasms
			\|-
			\|Type
			\|Marginal zone lymphoma
			\|-
			\|Subtype(s)
			\|Paediatric nodal marginal zone lymphoma
			\|}

	==Definition / Description of Disease==		==Definition / Description of Disease==

Bailey.Glen at 22:07, 6 September 2024

2024-09-06T22:07:52Z

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Bailey.Glen at 19:36, 13 December 2023

2023-12-13T19:36:17Z

← Older revision		Revision as of 14:36, 13 December 2023
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	<blockquote class='blockedit'>{{Box-round\|title=HAEM5 Conversion Notes\|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Paediatric Nodal Marginal Zone Lymphoma]].		<blockquote class='blockedit'>{{Box-round\|title=HAEM5 Conversion Notes\|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Paediatric Nodal Marginal Zone Lymphoma]].
	}}</blockquote>		}}</blockquote>

			<span style="color:#0070C0">(General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples). Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see </span><u>[[Author_Instructions]]</u><span style="color:#0070C0"> and [[Frequently Asked Questions (FAQs)\|<u>FAQs</u>]] as well as contact your [[Leadership\|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>])</span>

	==Primary Author(s)*==		==Primary Author(s)*==