Compendium of Cancer Genome Aberrations

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HAEM5:Lymphoplasmacytic lymphoma (view source)

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<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-11-03. The original page can be found at [[HAEM4:Lymphoplasmacytic Lymphoma]].
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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:Lymphoplasmacytic Lymphoma]].
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Other relevent pages include: [[Waldenstrom Macroglobulinemia]]
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Other relevent pages include: [[HAEM4:Waldenstrom Macroglobulinemia]]
    
Note: author needs to coorelate with Waldenstrom Macroglobulinemia
 
Note: author needs to coorelate with Waldenstrom Macroglobulinemia
 
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<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>
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==Primary Author(s)*==
 
==Primary Author(s)*==
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__TOC__
 
__TOC__
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==Cancer Category/Type==
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==Cancer Category / Type==
    
Low-grade mature B-cell neoplasm<ref name=":0">Vardiman JW, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p232-235</ref><ref name=":2">{{Cite journal|last=Varettoni|first=Marzia|last2=Boveri|first2=Emanuela|last3=Zibellini|first3=Silvia|last4=Tedeschi|first4=Alessandra|last5=Candido|first5=Chiara|last6=Ferretti|first6=Virginia Valeria|last7=Rizzo|first7=Ettore|last8=Doni|first8=Elisa|last9=Merli|first9=Michele|date=2019-11|title=Clinical and molecular characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: A multicentric study of the Rete Ematologica Lombarda (REL) network|url=https://pubmed.ncbi.nlm.nih.gov/31378966|journal=American Journal of Hematology|volume=94|issue=11|pages=1193–1199|doi=10.1002/ajh.25600|issn=1096-8652|pmid=31378966}}</ref>
 
Low-grade mature B-cell neoplasm<ref name=":0">Vardiman JW, et al., (2017). Acute myeloid leukaemia with recurrent genetic abnormalities, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p232-235</ref><ref name=":2">{{Cite journal|last=Varettoni|first=Marzia|last2=Boveri|first2=Emanuela|last3=Zibellini|first3=Silvia|last4=Tedeschi|first4=Alessandra|last5=Candido|first5=Chiara|last6=Ferretti|first6=Virginia Valeria|last7=Rizzo|first7=Ettore|last8=Doni|first8=Elisa|last9=Merli|first9=Michele|date=2019-11|title=Clinical and molecular characteristics of lymphoplasmacytic lymphoma not associated with an IgM monoclonal protein: A multicentric study of the Rete Ematologica Lombarda (REL) network|url=https://pubmed.ncbi.nlm.nih.gov/31378966|journal=American Journal of Hematology|volume=94|issue=11|pages=1193–1199|doi=10.1002/ajh.25600|issn=1096-8652|pmid=31378966}}</ref>
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*Lymphoplasmacytic lymphoma (LPL) is a neoplasm of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and spleen, which does not fulfil the criteria for any of the other small B-cell lymphoid neoplasms, that can have plasmacytic differentiation. <ref name=":0" />
 
*Lymphoplasmacytic lymphoma (LPL) is a neoplasm of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells, usually involving bone marrow and spleen, which does not fulfil the criteria for any of the other small B-cell lymphoid neoplasms, that can have plasmacytic differentiation. <ref name=":0" />
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*Based on the presence of the bone marrow involvement and the presence of paraprotein, LPL is categorized into [[Waldenstrom Macroglobulinemia|Waldenström macroglobulinemia]]  and LPL of non-Waldenström macroglobulinemia. <ref name=":1">{{Cite journal|last=Wang|first=Wei|last2=Lin|first2=Pei|date=2020-01|title=Lymphoplasmacytic lymphoma and Waldenström macroglobulinaemia: clinicopathological features and differential diagnosis|url=https://pubmed.ncbi.nlm.nih.gov/31767130|journal=Pathology|volume=52|issue=1|pages=6–14|doi=10.1016/j.pathol.2019.09.009|issn=1465-3931|pmid=31767130}}</ref>
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*Based on the presence of the bone marrow involvement and the presence of paraprotein, LPL is categorized into [[HAEM5:Lymphoplasmacytic lymphoma|Waldenström macroglobulinemia]]  and LPL of non-Waldenström macroglobulinemia. <ref name=":1">{{Cite journal|last=Wang|first=Wei|last2=Lin|first2=Pei|date=2020-01|title=Lymphoplasmacytic lymphoma and Waldenström macroglobulinaemia: clinicopathological features and differential diagnosis|url=https://pubmed.ncbi.nlm.nih.gov/31767130|journal=Pathology|volume=52|issue=1|pages=6–14|doi=10.1016/j.pathol.2019.09.009|issn=1465-3931|pmid=31767130}}</ref>
 
*The majority of cases of LPL lymphoma are associated with IgM paraprotein secretion (Waldenström macroglobulinemia), with less than 5% of cases attributed to IgA, IgG, and nonsecreting cases of LPL. <ref>{{Cite journal|last=Hunter|first=Zachary R.|last2=Yang|first2=Guang|last3=Xu|first3=Lian|last4=Liu|first4=Xia|last5=Castillo|first5=Jorge J.|last6=Treon|first6=Steven P.|date=2017-03-20|title=Genomics, Signaling, and Treatment of Waldenström Macroglobulinemia|url=https://pubmed.ncbi.nlm.nih.gov/28294689|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=35|issue=9|pages=994–1001|doi=10.1200/JCO.2016.71.0814|issn=1527-7755|pmid=28294689}}</ref>
 
*The majority of cases of LPL lymphoma are associated with IgM paraprotein secretion (Waldenström macroglobulinemia), with less than 5% of cases attributed to IgA, IgG, and nonsecreting cases of LPL. <ref>{{Cite journal|last=Hunter|first=Zachary R.|last2=Yang|first2=Guang|last3=Xu|first3=Lian|last4=Liu|first4=Xia|last5=Castillo|first5=Jorge J.|last6=Treon|first6=Steven P.|date=2017-03-20|title=Genomics, Signaling, and Treatment of Waldenström Macroglobulinemia|url=https://pubmed.ncbi.nlm.nih.gov/28294689|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=35|issue=9|pages=994–1001|doi=10.1200/JCO.2016.71.0814|issn=1527-7755|pmid=28294689}}</ref>
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*The median age is in the seventh decade of life. <ref name=":0" /><ref name=":1" />
 
*The median age is in the seventh decade of life. <ref name=":0" /><ref name=":1" />
 
*Approximately 1000-1500 new cases are diagnosed yearly in the United States.
 
*Approximately 1000-1500 new cases are diagnosed yearly in the United States.
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*LPL associated with IgM ([[Waldenstrom Macroglobulinemia|Waldenström macroglobulinemia]]) is more commonly occurs in male patients, while LPL  with a non-IgM is reported more frequently in females. <ref name=":2" />
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*LPL associated with IgM ([[HAEM5:Lymphoplasmacytic lymphoma|Waldenström macroglobulinemia]]) is more commonly occurs in male patients, while LPL  with a non-IgM is reported more frequently in females. <ref name=":2" />
    
==Clinical Features==
 
==Clinical Features==
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*Rare involvement of CNS associated with Waldenström macroglobulinemia (known as [[Bing-Neel syndrome]]). Lymphoplasmacytic lymphoma may occur at sites typically involved by extranodal marginal zone lymphoma (MZL) of mucosal-associated lymphoid tissue (MALT lymphoma), such as the ocular adnexa. <ref name=":0" /><ref name=":1" />
 
*Rare involvement of CNS associated with Waldenström macroglobulinemia (known as [[Bing-Neel syndrome]]). Lymphoplasmacytic lymphoma may occur at sites typically involved by extranodal marginal zone lymphoma (MZL) of mucosal-associated lymphoid tissue (MALT lymphoma), such as the ocular adnexa. <ref name=":0" /><ref name=":1" />
 
*Patients with non-IgM LPL are more likely to present with extramedullary involvement such as lymphadenopathy, splenomegaly, or extranodal disease. <ref name=":2" />
 
*Patients with non-IgM LPL are more likely to present with extramedullary involvement such as lymphadenopathy, splenomegaly, or extranodal disease. <ref name=":2" />
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*Bone marrow involvement is more common in patients with IgM-associated LPL ([[Waldenstrom Macroglobulinemia|Waldenström macroglobulinemia]]) when compared to non-IgM LPL. <ref name=":2" />
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*Bone marrow involvement is more common in patients with IgM-associated LPL ([[HAEM5:Lymphoplasmacytic lymphoma|Waldenström macroglobulinemia]]) when compared to non-IgM LPL. <ref name=":2" />
    
==Morphologic Features==
 
==Morphologic Features==
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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.}}<ref name=":0" /><ref name=":1" /></blockquote>
 
<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.}}<ref name=":0" /><ref name=":1" /></blockquote>
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==Individual Region Genomic Gain/Loss/LOH==
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==Individual Region Genomic Gain / Loss / LOH==
    
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==Gene Mutations (SNV/INDEL)==
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==Gene Mutations (SNV / INDEL)==
    
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<nowiki>*</nowiki>''Citation of this Page'': “Lymphoplasmacytic lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Lymphoplasmacytic_lymphoma</nowiki>.[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases L]]
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<nowiki>*</nowiki>''Citation of this Page'': “Lymphoplasmacytic lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Lymphoplasmacytic_lymphoma</nowiki>.
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[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases L]]
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