HAEM5:ALK-negative anaplastic large cell lymphoma - Revision history

Bailey.Glen at 17:09, 3 July 2025

2025-07-03T17:09:11Z

← Older revision		Revision as of 12:09, 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 21:28, 25 May 2025

2025-05-25T21:28:35Z

← Older revision		Revision as of 16:28, 25 May 2025
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	<nowiki></nowiki> These rearrangements are considered mutually exclusive; however, a single case with both ''DUSP22'' and ''TP63'' rearrangement has been described<ref name=":11" />. Can also be seen in a fraction of other PTCL.		<nowiki></nowiki> These rearrangements are considered mutually exclusive; however, a single case with both ''DUSP22'' and ''TP63'' rearrangement has been described<ref name=":11" />. Can also be seen in a fraction of other PTCL.
	*5-year overall survival > 90%		*5-year overall survival > 90%
	*~~'''~~Therapeutic Implications~~'''~~		*Therapeutic Implications
	**Multi-agent chemotherapy (CHOEP or CHOP-based) as first-line, with or without radiotherapy of involved site		**Multi-agent chemotherapy (CHOEP or CHOP-based) as first-line, with or without radiotherapy of involved site
	**High dose chemotherapy and autologous stem cell transplantation for remission		**High dose chemotherapy and autologous stem cell transplantation for remission
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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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	*Prevalence 19-26%		*Prevalence 19-26%
	\|-		\|-
	\|~~'''~~6q~~'''~~		\|6q
	\|~~'''~~Loss > CN-LOH~~'''~~		\|Loss > CN-LOH
	\|21		\|21
	\|		\|
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	\|		\|
	*Prevalence 35%		*Prevalence 35%
	*Genes affected: ~~'''~~''PRDM1~~'''~~, ATG5''		*Genes affected: ''PRDM1, ATG5''
	\|-		\|-
	\|10p		\|10p
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	\|17p		\|17p
	\|Loss		\|Loss
	\|~~'''~~13.3-p12~~'''~~		\|13.3-p12
	\|		\|
	\|No		\|No
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	19%		19%
	\|-		\|-
	\|~~'''~~6q~~'''~~		\|6q
	\|~~'''~~Loss > CN-LOH;~~'''~~		\|Loss > CN-LOH;
	See also below for somatic mutations		See also below for somatic mutations
	\|~~'''~~21~~'''~~		\|21
	\|~~'''~~PRDM1~~'''~~, ATG5		\|PRDM1, ATG5
	\|~~'''~~35%~~'''~~		\|35%
	\|-		\|-
	\|10p		\|10p
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	\|29%		\|29%
	\|-		\|-
	\|~~'''~~17p~~'''~~		\|17p
	\|~~'''~~Loss~~'''~~		\|Loss
	\|~~'''~~13.3-p12~~'''~~		\|13.3-p12
	\|~~'''~~TP53~~'''~~		\|TP53
	\|~~'''~~42%~~'''~~		\|42%
	\|}		\|}

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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''
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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!!Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)!!Prevalence (COSMIC / TCGA / Other)!!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)
	!Therapeutic Significance (Yes, No or Unknown)		!Therapeutic Significance (Yes, No or Unknown)
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	\|Positive (universal)\|\|CD30*, CD43 (almost universally)		\|Positive (universal)\|\|CD30*, CD43 (almost universally)
	\|-		\|-
	\|Negative (universal)\|\|~~'''~~ALK~~'''~~, ~~'''~~TP63~~'''~~, EBER, LMP-1		\|Negative (universal)\|\|ALK, TP63, EBER, LMP-1
	\|-		\|-
	\|Positive (frequent)		\|Positive (frequent)
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	!Finding!!Marker		!Finding!!Marker
	\|-		\|-
	\|Positive (universal)\|\|CD30*, CD43 (almost universally), ~~'''~~P63~~'''~~, CD4+ cases more common than CD8		\|Positive (universal)\|\|CD30*, CD43 (almost universally), P63, CD4+ cases more common than CD8
	\|-		\|-
	\|Negative (universal)\|\|~~'''~~ALK~~'''~~, EBER, LMP-1		\|Negative (universal)\|\|ALK, EBER, LMP-1
	\|-		\|-
	\|Positive (frequent)		\|Positive (frequent)
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	\|Positive (universal)\|\|CD30*, CD43 (almost universally), CD2, CD3, CD4+ cases more common than CD8, CD5, TIA1, granzyme B, perforin, EMA		\|Positive (universal)\|\|CD30*, CD43 (almost universally), CD2, CD3, CD4+ cases more common than CD8, CD5, TIA1, granzyme B, perforin, EMA
	\|-		\|-
	\|Negative (universal)\|\|~~'''~~ALK~~'''~~, ~~'''~~P63~~'''~~, EBER, LMP-1		\|Negative (universal)\|\|ALK, P63, EBER, LMP-1
	\|-		\|-
	\|Positive (common)		\|Positive (common)

Bailey.Glen at 20:04, 25 May 2025

2025-05-25T20:04:58Z

← Older revision		Revision as of 15:04, 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
	\|		\|Anaplastic large cell lymphoma, ALK-negative
	\|-		\|-
	\|Not Recommended		\|Not Recommended
	\|		\|N/A
	\|}		\|}

Bailey.Glen at 17:31, 24 March 2025

2025-03-24T17:31:44Z

← Older revision		Revision as of 12:31, 24 March 2025
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	{{DISPLAYTITLE:ALK-negative anaplastic large cell lymphoma}}		{{DISPLAYTITLE:ALK-negative anaplastic large cell 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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	Cedars-Sinai, Los Angeles, CA		Cedars-Sinai, Los Angeles, CA

	~~__TOC__~~

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

Bailey.Glen: /* Gene Mutations (SNV/INDEL) */

2025-02-14T01:26:47Z

Gene Mutations (SNV/INDEL)

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 ==Gene Mutations (SNV/INDEL)==
 {| class="wikitable sortable"
@@ Line 661: / Line 704: @@
 ----
 </blockquote>
 ==Epigenomic Alterations==

Bailey.Glen: /* Characteristic Chromosomal or Other Global Mutational Patterns */

2025-02-14T01:25:13Z

Characteristic Chromosomal or Other Global Mutational Patterns

← Older revision		Revision as of 20:25, 13 February 2025
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	</blockquote>		</blockquote>

	~~==Characteristic Chromosomal or Other Global Mutational Patterns==~~
	==Characteristic Chromosomal or Other Global Mutational Patterns==		==Characteristic Chromosomal or Other Global Mutational Patterns==
	Put your text here and fill in the table <span style="color:#0070C0">(I''nstructions: Included in this category are alterations such as hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis; microsatellite instability; homologous recombination deficiency; mutational signature pattern; etc. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>		Put your text here and fill in the table <span style="color:#0070C0">(I''nstructions: Included in this category are alterations such as hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis; microsatellite instability; homologous recombination deficiency; mutational signature pattern; etc. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>

Bailey.Glen: /* Characteristic Chromosomal or Other Global Mutational Patterns */

2025-02-14T01:24:59Z

Characteristic Chromosomal or Other Global Mutational Patterns

← Older revision		Revision as of 20:24, 13 February 2025
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	==Characteristic Chromosomal or Other Global Mutational Patterns==		==Characteristic Chromosomal or Other Global Mutational Patterns==
			==Characteristic Chromosomal or Other Global Mutational Patterns==
	Put your text here <span style="color:#0070C0">(''~~EXAMPLE PATTERNS~~: hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis. Do not delete table.'')</span>		Put your text here and fill in the table <span style="color:#0070C0">(I''nstructions: Included in this category are alterations such as hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis; microsatellite instability; homologous recombination deficiency; mutational signature pattern; etc. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Please include references throughout the table. Do not delete the table.'')</span>

	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
	!Chromosomal Pattern		!Chromosomal Pattern
	!~~Diagnostic Significance (Yes~~, No or ~~Unknown~~)		!Molecular Pathogenesis
	!Prognostic Significance ~~(Yes~~, ~~No or Unknown)~~		!'''Prevalence -'''
	!~~Therapeutic~~ Significance (Yes, No ~~or Unknown~~)		'''Common >20%, Recurrent 5-20% or Rare <5% (Disease)'''
	!Notes		!'''Diagnostic, Prognostic, and Therapeutic Significance - D, P, T'''
			!'''Established Clinical Significance Per Guidelines - Yes or No (Source)'''
			!'''Clinical Relevance Details/Other Notes'''
			\|-
			\|<span class="blue-text">EXAMPLE:</span>
			Co-deletion of 1p and 18q
			\|<span class="blue-text">EXAMPLE:</span> See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
			\|<span class="blue-text">EXAMPLE:</span> Common (Oligodendroglioma)
			\|<span class="blue-text">EXAMPLE:</span> D, P
			\|
			\|
			\|-
			\|<span class="blue-text">EXAMPLE:</span>
			Microsatellite instability - hypermutated
			\|
			\|<span class="blue-text">EXAMPLE:</span> Common (Endometrial carcinoma)
			\|<span class="blue-text">EXAMPLE:</span> P, T
			\|
			\|
	\|-		\|-
	\|~~N/A~~		\|
	\|~~N/A~~		\|
	\|~~N/A~~		\|
	\|~~N/A~~		\|
	\|~~N/A~~		\|
			\|
	\|}		\|}

	<blockquote class="blockedit">{{Box-round\|title=v4:Characteristic Chromosomal Aberrations / Patterns\|The content below was from the old template. Please incorporate above.}}</blockquote>		<blockquote class="blockedit">{{Box-round\|title=v4:Characteristic Chromosomal Aberrations / Patterns\|The content below was from the old template. Please incorporate above.}}</blockquote>

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	----		----
	</blockquote>		</blockquote>

	==Gene Mutations (SNV/INDEL)==		==Gene Mutations (SNV/INDEL)==

Bailey.Glen: /* Individual Region Genomic Gain/Loss/LOH */

2025-02-14T01:24:02Z

Individual Region Genomic Gain/Loss/LOH

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 ==Individual Region Genomic Gain/Loss/LOH==
 {| class="wikitable sortable"
@@ Line 436: / Line 487: @@
 ----
 </blockquote>
 ==Characteristic Chromosomal or Other Global Mutational Patterns==

Bailey.Glen: /* Gene Rearrangements */

2025-02-14T01:23:16Z

Gene Rearrangements

@@ Line 68: / Line 68: @@
 ==Gene Rearrangements==
-Put your text here and fill in the table
+Both balanced and unbalanced forms are observed by FISH (add references).
 {| class="wikitable sortable"
@@ Line 197: / Line 253: @@
 ----
 </blockquote>
 ==Individual Region Genomic Gain/Loss/LOH==

Jennelleh at 22:29, 13 February 2025

2025-02-13T22:29:15Z

← Older revision		Revision as of 17:29, 13 February 2025
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	==Additional Information==		==Additional Information==
	This disease is defined/characterized as detailed below:		This disease is <u>defined/characterized</u> as detailed below:

	Anaplastic large cell lymphomas (ALCL), [[ALK]]-negative, is a CD30+ T-cell lymphoma that is morphologically and immunophenotypically indistinguishable (but lacks ALK protein expression) from [[HAEM5:ALK-positive anaplastic large cell lymphoma\|ALK(+) ALCL]]<ref name=":9">{{Cite journal\|last=Ad\|first=Attygalle\|last2=J\|first2=Cabeçadas\|last3=P\|first3=Gaulard\|last4=Es\|first4=Jaffe\|last5=D\|first5=de Jong\|last6=Yh\|first6=Ko\|last7=J\|first7=Said\|last8=W\|first8=Klapper\|date=2014\|title=Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward - report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology\|url=https://pubmed.ncbi.nlm.nih.gov/24128129/\|language=en\|doi=10.1111/his.12251\|pmc=PMC6364972\|pmid=24128129}}</ref><ref name=":7" />		Anaplastic large cell lymphomas (ALCL), [[ALK]]-negative, is a CD30+ T-cell lymphoma that is morphologically and immunophenotypically indistinguishable (but lacks ALK protein expression) from [[HAEM5:ALK-positive anaplastic large cell lymphoma\|ALK(+) ALCL]]<ref name=":9">{{Cite journal\|last=Ad\|first=Attygalle\|last2=J\|first2=Cabeçadas\|last3=P\|first3=Gaulard\|last4=Es\|first4=Jaffe\|last5=D\|first5=de Jong\|last6=Yh\|first6=Ko\|last7=J\|first7=Said\|last8=W\|first8=Klapper\|date=2014\|title=Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward - report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology\|url=https://pubmed.ncbi.nlm.nih.gov/24128129/\|language=en\|doi=10.1111/his.12251\|pmc=PMC6364972\|pmid=24128129}}</ref><ref name=":7" />
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	***ERBB4 expression (~25%): mutually exclusive with other rearrangements (TP63, DUSP22, ROS or TYK translocations)<ref name=":4" />		***ERBB4 expression (~25%): mutually exclusive with other rearrangements (TP63, DUSP22, ROS or TYK translocations)<ref name=":4" />

	The epidemiology/prevalence of this disease is detailed below:		The <u>epidemiology/prevalence</u> of this disease is detailed below:

	*More common in adults than children (peak incidence 6th decade of life)<ref name=":1">{{Cite journal\|last=G\|first=Hapgood\|last2=Kj\|first2=Savage\|date=2015\|title=The biology and management of systemic anaplastic large cell lymphoma\|url=https://pubmed.ncbi.nlm.nih.gov/25869285/\|language=en\|pmid=25869285}}</ref>		*More common in adults than children (peak incidence 6th decade of life)<ref name=":1">{{Cite journal\|last=G\|first=Hapgood\|last2=Kj\|first2=Savage\|date=2015\|title=The biology and management of systemic anaplastic large cell lymphoma\|url=https://pubmed.ncbi.nlm.nih.gov/25869285/\|language=en\|pmid=25869285}}</ref>
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	*M:F 1.5:1<ref name=":1" />		*M:F 1.5:1<ref name=":1" />

	The clinical features of this disease are detailed below:		The <u>clinical features</u> of this disease are detailed below:

	Signs and symptoms - B-symptoms (weight loss, fever, night sweats)<ref name=":1" />; Peripheral and/or Lymphadenopathy<ref name=":1" />; Most patients present with advanced stage disease<ref name=":1" />		Signs and symptoms - B-symptoms (weight loss, fever, night sweats)<ref name=":1" />; Peripheral and/or Lymphadenopathy<ref name=":1" />; Most patients present with advanced stage disease<ref name=":1" />
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	Laboratory findings - Not specific		Laboratory findings - Not specific

	The sites of involvement of this disease are detailed below:		The <u>sites of involvement</u> of this disease are detailed below:

	*Nodal (predominantly abdominal lymphadenopathy) in a sinusoidal pattern		*Nodal (predominantly abdominal lymphadenopathy) in a sinusoidal pattern
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	**If involving the skin or GI tract, cases must be distinguished from primary cutaneous ALCL or CD30+ enteropathy-associated/other intestinal T-cell lymphomas, respectively		**If involving the skin or GI tract, cases must be distinguished from primary cutaneous ALCL or CD30+ enteropathy-associated/other intestinal T-cell lymphomas, respectively

	The morphologic features of this disease are detailed below:		The <u>morphologic features</u> of this disease are detailed below:

	*Tissue effacement by cohesive sheets of large, pleomorphic neoplastic cells, with or without prominent nucleoli, with varying proportions of hallmark cells		*Tissue effacement by cohesive sheets of large, pleomorphic neoplastic cells, with or without prominent nucleoli, with varying proportions of hallmark cells
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	*Intrasinusoidal growth pattern seen in cases with preserved nodal architecture		*Intrasinusoidal growth pattern seen in cases with preserved nodal architecture

	The immunophenotype of this disease is detailed below:		The <u>immunophenotype</u> of this disease is detailed below:

	Immunohistochemical patterns vary by subtype<ref name=":1" /><ref>{{Cite journal\|last=M\|first=Herling\|last2=Gz\|first2=Rassidakis\|last3=D\|first3=Jones\|last4=A\|first4=Schmitt-Graeff\|last5=Ah\|first5=Sarris\|last6=Lj\|first6=Medeiros\|date=2004\|title=Absence of Epstein-Barr virus in anaplastic large cell lymphoma: a study of 64 cases classified according to World Health Organization criteria\|url=https://pubmed.ncbi.nlm.nih.gov/15116326/\|language=en\|pmid=15116326}}</ref><ref name=":0" />		Immunohistochemical patterns vary by subtype<ref name=":1" /><ref>{{Cite journal\|last=M\|first=Herling\|last2=Gz\|first2=Rassidakis\|last3=D\|first3=Jones\|last4=A\|first4=Schmitt-Graeff\|last5=Ah\|first5=Sarris\|last6=Lj\|first6=Medeiros\|date=2004\|title=Absence of Epstein-Barr virus in anaplastic large cell lymphoma: a study of 64 cases classified according to World Health Organization criteria\|url=https://pubmed.ncbi.nlm.nih.gov/15116326/\|language=en\|pmid=15116326}}</ref><ref name=":0" />