HAEM4:Splenic Marginal Zone Lymphoma: Difference between revisions

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<blockquote class='blockedit'>{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].

<blockquote class="blockedit">{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].

}}</blockquote>

==Primary Author(s)*==

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==Cancer Category/Type==

*[[Mature B-Cell Neoplasms]]

*[[HAEM4:Mature B-Cell Neoplasms]]

==Cancer Sub-Classification / Subtype==

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==Definition / Description of Disease==

*Marginal zone lymphomas (MZL) make up 7% of non-Hodgkin lymphomas. The three subtypes are: MALT (70%), splenic (20%), and nodal (10%). '''Splenic marginal zone lymphoma (SMZL)''' is an Indolent mature B-cell neoplasm of adults

*Indolent mature B-cell neoplasm of adults involving the spleen, blood, and bone marrow

*Splenic white pulp effacement by small lymphocytes and pale marginal zone involved by larger cells

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*Males ~ Females

==Clinical Features<ref name=":2" /><ref name=":6" />==

==Clinical Features<ref name=":2" /><ref name=":6" /><small><sup>[3]</sup></small>==

'''Signs & Symptoms'''

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|7q31-32||Loss (heterozygous)

|Unknown; possible haploinsufficiency of IRF5 tumor suppressor<ref>{{Cite journal|last=Fresquet|first=Vicente|last2=Robles|first2=Eloy F.|last3=Parker|first3=Anton|last4=Martinez-Useros|first4=Javier|last5=Mena|first5=Maria|last6=Malumbres|first6=Raquel|last7=Agirre|first7=Xabier|last8=Catarino|first8=Susana|last9=Arteta|first9=David|date=2012|title=High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma|url=http://doi.wiley.com/10.1111/j.1365-2141.2012.09226.x|journal=British Journal of Haematology|language=en|volume=158|issue=6|pages=712–726|doi=10.1111/j.1365-2141.2012.09226.x}}</ref>||26–45%<ref name=":2">{{Cite journal|last=Salido|first=Marta|last2=Baró|first2=Cristina|last3=Oscier|first3=David|last4=Stamatopoulos|first4=Kostas|last5=Dierlamm|first5=Judith|last6=Matutes|first6=Estela|last7=Traverse-Glehen|first7=Alexandra|last8=Berger|first8=Francoise|last9=Felman|first9=Pascale|date=2010|title=Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group|url=https://ashpublications.org/blood/article/116/9/1479/103841/Cytogenetic-aberrations-and-their-prognostic-value|journal=Blood|language=en|volume=116|issue=9|pages=1479–1488|doi=10.1182/blood-2010-02-267476|issn=0006-4971}}</ref><ref name=":3">{{Cite journal|last=Baró|first=Cristina|last2=Salido|first2=Marta|last3=Espinet|first3=Blanca|last4=Astier|first4=Laura|last5=Domingo|first5=Alicia|last6=Granada|first6=Isabel|last7=Millà|first7=Fuensanta|last8=Carrió|first8=Ana|last9=Costa|first9=Dolors|date=2008|title=New chromosomal alterations in a series of 23 splenic marginal zone lymphoma patients revealed by Spectral Karyotyping (SKY)|url=https://linkinghub.elsevier.com/retrieve/pii/S0145212607003505|journal=Leukemia Research|language=en|volume=32|issue=5|pages=727–736|doi=10.1016/j.leukres.2007.09.012}}</ref><ref name=":4">{{Cite journal|last=Gruszka-Westwood|first=Alicja M.|last2=Hamoudi|first2=Rifat|last3=Osborne|first3=Lucy|last4=Matutes|first4=Estella|last5=Catovsky|first5=Daniel|date=2003|title=Deletion mapping on the long arm of chromosome 7 in splenic lymphoma with villous lymphocytes|url=http://doi.wiley.com/10.1002/gcc.10142|journal=Genes, Chromosomes and Cancer|language=en|volume=36|issue=1|pages=57–69|doi=10.1002/gcc.10142|issn=1045-2257}}</ref>

|-

|17p13 (TP53)

|Loss

|

|3%–17%

|-

|3/3q||Gain (trisomy)

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|BRAF mutations

|Diagnostic (exclusion)

|Present in [[Hairy ~~Cell Leukemia~~|hairy cell leukemia (HCL)]] and absent in SMZL<ref>{{Cite journal|last=Naseem|first=Shano|last2=Gupta|first2=Ojas|last3=Binota|first3=Jogeshwar|last4=Varma|first4=Neelam|last5=Varma|first5=Subhash|last6=Malhotra|first6=Pankaj|date=2020|title=BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system|url=http://link.springer.com/10.1007/s11033-020-05509-0|journal=Molecular Biology Reports|language=en|doi=10.1007/s11033-020-05509-0|issn=0301-4851}}</ref>

|Present in [[HAEM5:Hairy cell leukaemia|hairy cell leukemia (HCL)]] and absent in SMZL<ref>{{Cite journal|last=Naseem|first=Shano|last2=Gupta|first2=Ojas|last3=Binota|first3=Jogeshwar|last4=Varma|first4=Neelam|last5=Varma|first5=Subhash|last6=Malhotra|first6=Pankaj|date=2020|title=BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system|url=http://link.springer.com/10.1007/s11033-020-05509-0|journal=Molecular Biology Reports|language=en|doi=10.1007/s11033-020-05509-0|issn=0301-4851}}</ref>

|-

|MYD88 mutations

|Diagnostic (exclusion)

|Present in [[Lymphoplasmacytic ~~Lymphoma~~|lymphoplasmacytic lymphoma (LPL)]] and rare but not absent in SMZL

|Present in [[HAEM5:Lymphoplasmacytic lymphoma|lymphoplasmacytic lymphoma (LPL)]] and rare but not absent in SMZL

|-

|t(11;14)(q13;q32)/IGH-CCND1*

|Diagnostic (exclusion)

|Present in [[Mantle ~~Cell Lymphoma~~|mantle cell lymphoma (MCL)]] and absent in SMZL

|Present in [[HAEM5:Mantle cell lymphoma|mantle cell lymphoma (MCL)]] and absent in SMZL

|-

|t(14;18)(q32;q21)/IGH-BCL2

|Diagnostic (exclusion)

|Present in [[Follicular ~~Lymphoma~~|follicular lymphoma (FL)]] and rare but not absent in SMZL<ref name=":1" />

|Present in [[HAEM5:Follicular lymphoma|follicular lymphoma (FL)]] and rare but not absent in SMZL<ref name=":1" />

|-

|t(11;18)(q21;q21)/BIRC3-MALT1

|Diagnostic (exclusion)

|Present in [[Extranodal ~~Marginal Zone Lymphoma~~ of ~~Mucosa~~-~~Associated Lymphoid Tissue (MALT Lymphoma)~~|MALT lymphoma]] and absent in SMZL

|Present in [[HAEM5:Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue|MALT lymphoma]] and absent in SMZL

|-

|t(14;18)(q32;q21)/IGH-MALT1

|Diagnostic (exclusion)

|Present in [[Extranodal ~~Marginal Zone Lymphoma~~ of ~~Mucosa~~-~~Associated Lymphoid Tissue (MALT Lymphoma)~~|MALT lymphoma]] and absent in SMZL<ref>{{Cite journal|last=Streubel|first=Berthold|last2=Lamprecht|first2=Andrea|last3=Dierlamm|first3=Judith|last4=Cerroni|first4=Lorenzo|last5=Stolte|first5=Manfred|last6=Ott|first6=German|last7=Raderer|first7=Markus|last8=Chott|first8=Andreas|date=2003|title=T(14;18)(q32;q21) involving IGH andMALT1 is a frequent chromosomal aberration in MALT lymphoma|url=https://ashpublications.org/blood/article/101/6/2335/106539/T1418q32q21-involving-IGH-andMALT1-is-a-frequent|journal=Blood|language=en|volume=101|issue=6|pages=2335–2339|doi=10.1182/blood-2002-09-2963|issn=1528-0020}}</ref>

|Present in [[HAEM5:Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue|MALT lymphoma]] and absent in SMZL<ref>{{Cite journal|last=Streubel|first=Berthold|last2=Lamprecht|first2=Andrea|last3=Dierlamm|first3=Judith|last4=Cerroni|first4=Lorenzo|last5=Stolte|first5=Manfred|last6=Ott|first6=German|last7=Raderer|first7=Markus|last8=Chott|first8=Andreas|date=2003|title=T(14;18)(q32;q21) involving IGH andMALT1 is a frequent chromosomal aberration in MALT lymphoma|url=https://ashpublications.org/blood/article/101/6/2335/106539/T1418q32q21-involving-IGH-andMALT1-is-a-frequent|journal=Blood|language=en|volume=101|issue=6|pages=2335–2339|doi=10.1182/blood-2002-09-2963|issn=1528-0020}}</ref>

|-

|t(1;14)(p22;q32)/IGH-BCL10

|Diagnostic (exclusion)

|Present in [[Extranodal ~~Marginal Zone Lymphoma~~ of ~~Mucosa~~-~~Associated Lymphoid Tissue (MALT Lymphoma)~~|MALT lymphoma]] and absent in SMZL

|Present in [[HAEM5:Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue|MALT lymphoma]] and absent in SMZL

|}

<nowiki>*</nowiki>Cases previously reported as SMZL with IGH-CCND1 fusion should now be classified as MCL

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==Links==

*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]

*[[HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]

*[[Splenic ~~Diffuse Red Pulp Small~~ B-cell ~~Lymphoma~~]]

*[[HAEM5:Splenic diffuse red pulp small B-cell lymphoma]]

==References==

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==Notes==

<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page. If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage). Additional global feedback or concerns are also welcome.

[[Category:HAEM4]] [[Category:DISEASE]]

[[Category:HAEM4]]

[[Category:DISEASE]]

@@ Line 2: / Line 2: @@
-<blockquote class='blockedit'>{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].
+<blockquote class="blockedit">{{Box-round|title=PREVIOUS EDITION|This page from the 4th edition of Haematolymphoid Tumours is being updated. See 5th edition [[HAEM5:Table_of_Contents|Table of Contents]].
 }}</blockquote>
 ==Primary Author(s)*==
@@ Line 11: / Line 11: @@
 ==Cancer Category/Type==
-*[[Mature B-Cell Neoplasms]]
+*[[HAEM4:Mature B-Cell Neoplasms]]
 ==Cancer Sub-Classification / Subtype==
@@ Line 19: / Line 19: @@
 ==Definition / Description of Disease==
+*Marginal zone lymphomas (MZL) make up 7% of non-Hodgkin lymphomas. The three subtypes are: MALT (70%), splenic (20%), and nodal (10%). '''Splenic marginal zone lymphoma (SMZL)''' is an Indolent mature B-cell neoplasm of adults
 *Indolent mature B-cell neoplasm of adults involving the spleen, blood, and bone marrow
 *Splenic white pulp effacement by small lymphocytes and pale marginal zone involved by larger cells
@@ Line 36: / Line 37: @@
 *Males ~ Females
-==Clinical Features<ref name=":2" /><ref name=":6" />==
+==Clinical Features<ref name=":2" /><ref name=":6" /><small><sup>[3]</sup></small>==
 '''Signs & Symptoms'''
@@ Line 105: / Line 106: @@
 |7q31-32||Loss (heterozygous)
 |Unknown; possible haploinsufficiency of IRF5 tumor suppressor<ref>{{Cite journal|last=Fresquet|first=Vicente|last2=Robles|first2=Eloy F.|last3=Parker|first3=Anton|last4=Martinez-Useros|first4=Javier|last5=Mena|first5=Maria|last6=Malumbres|first6=Raquel|last7=Agirre|first7=Xabier|last8=Catarino|first8=Susana|last9=Arteta|first9=David|date=2012|title=High-throughput sequencing analysis of the chromosome 7q32 deletion reveals IRF5 as a potential tumour suppressor in splenic marginal-zone lymphoma|url=http://doi.wiley.com/10.1111/j.1365-2141.2012.09226.x|journal=British Journal of Haematology|language=en|volume=158|issue=6|pages=712–726|doi=10.1111/j.1365-2141.2012.09226.x}}</ref>||26–45%<ref name=":2">{{Cite journal|last=Salido|first=Marta|last2=Baró|first2=Cristina|last3=Oscier|first3=David|last4=Stamatopoulos|first4=Kostas|last5=Dierlamm|first5=Judith|last6=Matutes|first6=Estela|last7=Traverse-Glehen|first7=Alexandra|last8=Berger|first8=Francoise|last9=Felman|first9=Pascale|date=2010|title=Cytogenetic aberrations and their prognostic value in a series of 330 splenic marginal zone B-cell lymphomas: a multicenter study of the Splenic B-Cell Lymphoma Group|url=https://ashpublications.org/blood/article/116/9/1479/103841/Cytogenetic-aberrations-and-their-prognostic-value|journal=Blood|language=en|volume=116|issue=9|pages=1479–1488|doi=10.1182/blood-2010-02-267476|issn=0006-4971}}</ref><ref name=":3">{{Cite journal|last=Baró|first=Cristina|last2=Salido|first2=Marta|last3=Espinet|first3=Blanca|last4=Astier|first4=Laura|last5=Domingo|first5=Alicia|last6=Granada|first6=Isabel|last7=Millà|first7=Fuensanta|last8=Carrió|first8=Ana|last9=Costa|first9=Dolors|date=2008|title=New chromosomal alterations in a series of 23 splenic marginal zone lymphoma patients revealed by Spectral Karyotyping (SKY)|url=https://linkinghub.elsevier.com/retrieve/pii/S0145212607003505|journal=Leukemia Research|language=en|volume=32|issue=5|pages=727–736|doi=10.1016/j.leukres.2007.09.012}}</ref><ref name=":4">{{Cite journal|last=Gruszka-Westwood|first=Alicja M.|last2=Hamoudi|first2=Rifat|last3=Osborne|first3=Lucy|last4=Matutes|first4=Estella|last5=Catovsky|first5=Daniel|date=2003|title=Deletion mapping on the long arm of chromosome 7 in splenic lymphoma with villous lymphocytes|url=http://doi.wiley.com/10.1002/gcc.10142|journal=Genes, Chromosomes and Cancer|language=en|volume=36|issue=1|pages=57–69|doi=10.1002/gcc.10142|issn=1045-2257}}</ref>
+|-
+|17p13 (TP53)
+|Loss
+|
+|3%–17%
 |-
 |3/3q||Gain (trisomy)
@@ Line 230: / Line 236: @@
 |BRAF mutations
 |Diagnostic (exclusion)
-|Present in [[Hairy Cell Leukemia|hairy cell leukemia (HCL)]] and absent in SMZL<ref>{{Cite journal|last=Naseem|first=Shano|last2=Gupta|first2=Ojas|last3=Binota|first3=Jogeshwar|last4=Varma|first4=Neelam|last5=Varma|first5=Subhash|last6=Malhotra|first6=Pankaj|date=2020|title=BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system|url=http://link.springer.com/10.1007/s11033-020-05509-0|journal=Molecular Biology Reports|language=en|doi=10.1007/s11033-020-05509-0|issn=0301-4851}}</ref>
+|Present in [[HAEM5:Hairy cell leukaemia|hairy cell leukemia (HCL)]] and absent in SMZL<ref>{{Cite journal|last=Naseem|first=Shano|last2=Gupta|first2=Ojas|last3=Binota|first3=Jogeshwar|last4=Varma|first4=Neelam|last5=Varma|first5=Subhash|last6=Malhotra|first6=Pankaj|date=2020|title=BRAF V600E mutation detection in hairy cell leukemia-utility of archival DNA from bone marrow aspirate/imprint smear and amplification refractory mutation system|url=http://link.springer.com/10.1007/s11033-020-05509-0|journal=Molecular Biology Reports|language=en|doi=10.1007/s11033-020-05509-0|issn=0301-4851}}</ref>
 |-
 |MYD88 mutations
 |Diagnostic (exclusion)
-|Present in [[Lymphoplasmacytic Lymphoma|lymphoplasmacytic lymphoma (LPL)]] and rare but not absent in SMZL
+|Present in [[HAEM5:Lymphoplasmacytic lymphoma|lymphoplasmacytic lymphoma (LPL)]] and rare but not absent in SMZL
 |-
 |t(11;14)(q13;q32)/IGH-CCND1*
 |Diagnostic (exclusion)
-|Present in [[Mantle Cell Lymphoma|mantle cell lymphoma (MCL)]] and absent in SMZL
+|Present in [[HAEM5:Mantle cell lymphoma|mantle cell lymphoma (MCL)]] and absent in SMZL
 |-
 |t(14;18)(q32;q21)/IGH-BCL2
 |Diagnostic (exclusion)
-|Present in [[Follicular Lymphoma|follicular lymphoma (FL)]] and rare but not absent in SMZL<ref name=":1" />
+|Present in [[HAEM5:Follicular lymphoma|follicular lymphoma (FL)]] and rare but not absent in SMZL<ref name=":1" />
 |-
 |t(11;18)(q21;q21)/BIRC3-MALT1
 |Diagnostic (exclusion)
-|Present in [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|MALT lymphoma]] and absent in SMZL
+|Present in [[HAEM5:Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue|MALT lymphoma]] and absent in SMZL
 |-
 |t(14;18)(q32;q21)/IGH-MALT1
 |Diagnostic (exclusion)
-|Present in [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|MALT lymphoma]] and absent in SMZL<ref>{{Cite journal|last=Streubel|first=Berthold|last2=Lamprecht|first2=Andrea|last3=Dierlamm|first3=Judith|last4=Cerroni|first4=Lorenzo|last5=Stolte|first5=Manfred|last6=Ott|first6=German|last7=Raderer|first7=Markus|last8=Chott|first8=Andreas|date=2003|title=T(14;18)(q32;q21) involving IGH andMALT1 is a frequent chromosomal aberration in MALT lymphoma|url=https://ashpublications.org/blood/article/101/6/2335/106539/T1418q32q21-involving-IGH-andMALT1-is-a-frequent|journal=Blood|language=en|volume=101|issue=6|pages=2335–2339|doi=10.1182/blood-2002-09-2963|issn=1528-0020}}</ref>
+|Present in [[HAEM5:Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue|MALT lymphoma]] and absent in SMZL<ref>{{Cite journal|last=Streubel|first=Berthold|last2=Lamprecht|first2=Andrea|last3=Dierlamm|first3=Judith|last4=Cerroni|first4=Lorenzo|last5=Stolte|first5=Manfred|last6=Ott|first6=German|last7=Raderer|first7=Markus|last8=Chott|first8=Andreas|date=2003|title=T(14;18)(q32;q21) involving IGH andMALT1 is a frequent chromosomal aberration in MALT lymphoma|url=https://ashpublications.org/blood/article/101/6/2335/106539/T1418q32q21-involving-IGH-andMALT1-is-a-frequent|journal=Blood|language=en|volume=101|issue=6|pages=2335–2339|doi=10.1182/blood-2002-09-2963|issn=1528-0020}}</ref>
 |-
 |t(1;14)(p22;q32)/IGH-BCL10
 |Diagnostic (exclusion)
-|Present in [[Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma)|MALT lymphoma]] and absent in SMZL
+|Present in [[HAEM5:Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue|MALT lymphoma]] and absent in SMZL
 |}
 <nowiki>*</nowiki>Cases previously reported as SMZL with IGH-CCND1 fusion should now be classified as MCL
@@ Line 268: / Line 274: @@
 ==Links==
-*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
+*[[HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
-*[[Splenic Diffuse Red Pulp Small B-cell Lymphoma]]
+*[[HAEM5:Splenic diffuse red pulp small B-cell lymphoma]]
 ==References==
@@ Line 280: / Line 286: @@
 ==Notes==
 <nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome.
-[[Category:HAEM4]] [[Category:DISEASE]]
+[[Category:HAEM4]]
+[[Category:DISEASE]]