Compendium of Cancer Genome Aberrations

HAEM4:Lymphoplasmacytic Lymphoma: Difference between revisions

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{{DISPLAYTITLE:Lymphoplasmacytic Lymphoma}}
<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)*==
==Primary Author(s)*==


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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" />
*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>
*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.
*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" />
*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<ref name=":0" /><ref name=":1" />==
==Clinical Features<ref name=":0" /><ref name=":1" />==
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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" />
*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" />
*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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==Notes==
==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.
<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]]
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