HAEM5:Burkitt lymphoma - Revision history

Richard.Glen at 18:37, 3 February 2026

2026-02-03T18:37:45Z

Richard.Glen: Removed redirect to BRST5:Burkitt lymphoma

2026-02-03T18:35:56Z

Removed redirect to BRST5:Burkitt lymphoma

← Older revision		Revision as of 13:35, 3 February 2026
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	~~#REDIRECT~~[[~~BRST5~~:~~Burkitt lymphoma~~]]		st-HAART eras\n\n[[Category:HAEM5]]\n[[Category:DISEASE]]\n[[Category:Diseases B]]"}}}

Richard.Glen: Redirected page to BRST5:Burkitt lymphoma

2026-02-03T18:30:12Z

Redirected page to BRST5:Burkitt lymphoma

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Bailey.Glen at 17:12, 3 July 2025

2025-07-03T17:12:08Z

← Older revision		Revision as of 12:12, 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 21:31, 25 May 2025

2025-05-25T21:31:35Z

← Older revision		Revision as of 16:31, 25 May 2025
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	!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence		!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence
	\|-		\|-
	\|t(8;14)(q24;q32)\|\|5'<nowiki/>''IGH'' / ~~3'''MYC~~''\|\|der(14)\|\|85%		\|t(8;14)(q24;q32)\|\|5'<nowiki/>''IGH'' / 3MYC''\|\|der(14)\|\|85%
	\|-		\|-
	\|t(8;22)(q24;q11)		\|t(8;22)(q24;q11)
	\|5'<nowiki/>''MYC'' / ~~3'''IGL~~''		\|5'<nowiki/>''MYC'' / 3IGL''
	\|der(8)		\|der(8)
	\|10%		\|10%
	\|-		\|-
	\|t(2;8)(p12;q24)\|\|5'<nowiki/>''MYC'' / ~~3'''IGK~~''\|\|der(8)\|\|5%		\|t(2;8)(p12;q24)\|\|5'<nowiki/>''MYC'' / 3IGK''\|\|der(8)\|\|5%
	\|}		\|}
	<blockquote class="blockedit">		<blockquote class="blockedit">
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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 20:07, 25 May 2025

2025-05-25T20:07:57Z

← Older revision		Revision as of 15:07, 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
	\|		\|Burkitt cell leukaemia; atypical Burkitt lymphoma; Burkitt-like lymphoma
	\|}		\|}

Bailey.Glen at 17:33, 24 March 2025

2025-03-24T17:33:50Z

← Older revision		Revision as of 12:33, 24 March 2025
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	{{DISPLAYTITLE:Burkitt lymphoma}}		{{DISPLAYTITLE:Burkitt 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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	Becky Leung, MBBS (Hons), BSc, Pathology Queensland		Becky Leung, MBBS (Hons), BSc, Pathology Queensland

	~~__TOC__~~

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

Bailey.Glen: Removed old template contents

2025-02-11T21:29:54Z

Removed old template contents

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Bailey.Glen at 18:43, 10 February 2025

2025-02-10T18:43:04Z

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Bailey.Glen at 18:15, 10 February 2025

2025-02-10T18:15:03Z

← Older revision		Revision as of 13:15, 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>

	Patients often present with bulky disease and high tumour burden, showing rapid clinical progression. The typically involved anatomical sites are different for the three subtypes. At presentation, 30% have localised (stage I or II) disease and 70% have advanced (stage III or IV) disease, according to the revised international paediatric non-Hodgkin lymphoma staging system.		Patients often present with bulky disease and high tumour burden, showing rapid clinical progression. The typically involved anatomical sites are different for the three subtypes. At presentation, 30% have localised (stage I or II) disease and 70% have advanced (stage III or IV) disease, according to the revised international paediatric non-Hodgkin lymphoma staging system.
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	Due to the high chemosensitivity of the tumour, treatment of Burkitt Lymphoma with chemotherapy can lead to rapid tumour cell death and an acute tumour lysis syndrome secondary to this.		Due to the high chemosensitivity of the tumour, treatment of Burkitt Lymphoma with chemotherapy can lead to rapid tumour cell death and an acute tumour lysis syndrome secondary to this.

			<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>
	The development of Burkitt lymphoma is dependent on the constitutive expression of the ''MYC'' proto-oncogene. The MYC encoded protein is a transcriptional regulator, controlling target genes involved in cell cycle regulation, metabolism and apoptosis. Dysregulation of ''MYC'' expression occurs due to juxtaposition of regulatory elements of the immunoglobulin loci, usually ''IGH'', but also ''IGL'' or ''IGK''. Overexpression of ''MYC'' correlates with increased cell survival. The different Burkitt lymphoma subtypes harbour diverse ''MYC'' and ''IGH'' locus breakpoints. In endemic Burkitt lymphoma, ''MYC'' usually breaks several hundred kilobases further upstream and ''IG'' usually breaks in the VDJ region. In contrast, most sporadic and immunodeficiency-associated Burkitt lymphoma have chromosomal breakpoints within exon 1 and the first intron of ''MYC'' and at the class switch region of ''IG''<ref>{{Cite journal\|last=Neri\|first=A.\|last2=Barriga\|first2=F.\|last3=Knowles\|first3=D. M.\|last4=Magrath\|first4=I. T.\|last5=Dalla-Favera\|first5=R.\|date=1988-04-01\|title=Different regions of the immunoglobulin heavy-chain locus are involved in chromosomal translocations in distinct pathogenetic forms of Burkitt lymphoma.\|url=http://dx.doi.org/10.1073/pnas.85.8.2748\|journal=Proceedings of the National Academy of Sciences\|volume=85\|issue=8\|pages=2748–2752\|doi=10.1073/pnas.85.8.2748\|issn=0027-8424}}</ref>. Although fluorescence ''in situ'' hydribisation (FISH) methods are well established in most pathology laboratories, no single probe set is able to cover all ''MYC'' breakpoints. In particular, distant breakpoints, complex rearrangements and cryptic insertions may be overlooked<ref>{{Cite journal\|last=Muñoz-Mármol\|first=Ana M\|last2=Sanz\|first2=Carolina\|last3=Tapia\|first3=Gustavo\|last4=Marginet\|first4=Ruth\|last5=Ariza\|first5=Aurelio\|last6=Mate\|first6=José L\|date=2013-09\|title=MYC status determination in aggressive B-cell lymphoma: the impact of FISH probe selection\|url=http://doi.wiley.com/10.1111/his.12178\|journal=Histopathology\|language=en\|volume=63\|issue=3\|pages=418–424\|doi=10.1111/his.12178}}</ref>. Hence, multiple FISH probe sets are required for comprehensive detection of clinically relevant ''MYC'' gene rearrangements.		The development of Burkitt lymphoma is dependent on the constitutive expression of the ''MYC'' proto-oncogene. The MYC encoded protein is a transcriptional regulator, controlling target genes involved in cell cycle regulation, metabolism and apoptosis. Dysregulation of ''MYC'' expression occurs due to juxtaposition of regulatory elements of the immunoglobulin loci, usually ''IGH'', but also ''IGL'' or ''IGK''. Overexpression of ''MYC'' correlates with increased cell survival. The different Burkitt lymphoma subtypes harbour diverse ''MYC'' and ''IGH'' locus breakpoints. In endemic Burkitt lymphoma, ''MYC'' usually breaks several hundred kilobases further upstream and ''IG'' usually breaks in the VDJ region. In contrast, most sporadic and immunodeficiency-associated Burkitt lymphoma have chromosomal breakpoints within exon 1 and the first intron of ''MYC'' and at the class switch region of ''IG''<ref>{{Cite journal\|last=Neri\|first=A.\|last2=Barriga\|first2=F.\|last3=Knowles\|first3=D. M.\|last4=Magrath\|first4=I. T.\|last5=Dalla-Favera\|first5=R.\|date=1988-04-01\|title=Different regions of the immunoglobulin heavy-chain locus are involved in chromosomal translocations in distinct pathogenetic forms of Burkitt lymphoma.\|url=http://dx.doi.org/10.1073/pnas.85.8.2748\|journal=Proceedings of the National Academy of Sciences\|volume=85\|issue=8\|pages=2748–2752\|doi=10.1073/pnas.85.8.2748\|issn=0027-8424}}</ref>. Although fluorescence ''in situ'' hydribisation (FISH) methods are well established in most pathology laboratories, no single probe set is able to cover all ''MYC'' breakpoints. In particular, distant breakpoints, complex rearrangements and cryptic insertions may be overlooked<ref>{{Cite journal\|last=Muñoz-Mármol\|first=Ana M\|last2=Sanz\|first2=Carolina\|last3=Tapia\|first3=Gustavo\|last4=Marginet\|first4=Ruth\|last5=Ariza\|first5=Aurelio\|last6=Mate\|first6=José L\|date=2013-09\|title=MYC status determination in aggressive B-cell lymphoma: the impact of FISH probe selection\|url=http://doi.wiley.com/10.1111/his.12178\|journal=Histopathology\|language=en\|volume=63\|issue=3\|pages=418–424\|doi=10.1111/his.12178}}</ref>. Hence, multiple FISH probe sets are required for comprehensive detection of clinically relevant ''MYC'' gene rearrangements.

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	\|t(2;8)(p12;q24)\|\|5'<nowiki/>''MYC'' / 3'''IGK''\|\|der(8)\|\|5%		\|t(2;8)(p12;q24)\|\|5'<nowiki/>''MYC'' / 3'''IGK''\|\|der(8)\|\|5%
	\|}		\|}
			<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>

	Deciphering the genomic landscape of Burkitt lymphoma provides additional molecular targets for new treatment regimens. Burkitt lymphoma is often curable using intensive chemotherapy treatments. However, these regimens may not be well tolerated by older individuals and further treatment options are required for those who exhibit refractory or relapsed disease.		Deciphering the genomic landscape of Burkitt lymphoma provides additional molecular targets for new treatment regimens. Burkitt lymphoma is often curable using intensive chemotherapy treatments. However, these regimens may not be well tolerated by older individuals and further treatment options are required for those who exhibit refractory or relapsed disease.
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	Considerations are for inhibitors of PI3K signaling and downstream pathways, and inhibiting cyclin dependent kinase 4/6 to block cyclin D3 mediated cell cycle progression.		Considerations are for inhibitors of PI3K signaling and downstream pathways, and inhibiting cyclin dependent kinase 4/6 to block cyclin D3 mediated cell cycle progression.

			<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>

	Most often, Burkitt lymphoma is associated with a simple karyotype. However additional chromosomal abnormalities may also occur and play a role in disease progression, see the table below for the more commonly implicated cytogenetic abnormalities.		Most often, Burkitt lymphoma is associated with a simple karyotype. However additional chromosomal abnormalities may also occur and play a role in disease progression, see the table below for the more commonly implicated cytogenetic abnormalities.
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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: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>

	{\| class="wikitable sortable"		{\| class="wikitable sortable"
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	\|14%		\|14%
	\|}		\|}
			<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>

	''MYC'' is the most commonly mutated gene in Burkitt lymphoma, such variants lead to constitutive expression of ''MYC'', which drives cell survival. Aberrant somatic hypermutation is understood to be the major cause of ''MYC'' breakpoint formation and the presence of hypermutation in tandem with MYC rearrangement may be detectable if using sequencing methodologies.		''MYC'' is the most commonly mutated gene in Burkitt lymphoma, such variants lead to constitutive expression of ''MYC'', which drives cell survival. Aberrant somatic hypermutation is understood to be the major cause of ''MYC'' breakpoint formation and the presence of hypermutation in tandem with MYC rearrangement may be detectable if using sequencing methodologies.
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	The gene expression and micro-RNA expression profiles of Burkitt lymphoma are different from other lymphomas. The expression profiles of endemic and sporadic BL are also slightly different to each other. There may be grey zones where Burkitt lymphoma is difficult to distinguish from diffuse large B-cell lymphoma based on gene expression, hence expression should not be used as an independent diagnostic tool<ref>{{Cite journal\|last=Shannon-Lowe\|first=Claire\|last2=Rickinson\|first2=Alan B.\|last3=Bell\|first3=Andrew I.\|date=2017-10-19\|title=Epstein-Barr virus-associated lymphomas\|url=https://pubmed.ncbi.nlm.nih.gov/28893938\|journal=Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences\|volume=372\|issue=1732\|doi=10.1098/rstb.2016.0271\|issn=1471-2970\|pmc=5597738\|pmid=28893938}}</ref>.		The gene expression and micro-RNA expression profiles of Burkitt lymphoma are different from other lymphomas. The expression profiles of endemic and sporadic BL are also slightly different to each other. There may be grey zones where Burkitt lymphoma is difficult to distinguish from diffuse large B-cell lymphoma based on gene expression, hence expression should not be used as an independent diagnostic tool<ref>{{Cite journal\|last=Shannon-Lowe\|first=Claire\|last2=Rickinson\|first2=Alan B.\|last3=Bell\|first3=Andrew I.\|date=2017-10-19\|title=Epstein-Barr virus-associated lymphomas\|url=https://pubmed.ncbi.nlm.nih.gov/28893938\|journal=Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences\|volume=372\|issue=1732\|doi=10.1098/rstb.2016.0271\|issn=1471-2970\|pmc=5597738\|pmid=28893938}}</ref>.

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