HAEM5:Lymphoplasmacytic lymphoma: Difference between revisions
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<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023- | <blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|This page was converted to the new template on 2023-12-04. The original page can be found at [[HAEM4:Lymphoplasmacytic Lymphoma]]. | ||
Other relevent pages include: [[Waldenstrom Macroglobulinemia]] | Other relevent pages include: [[Waldenstrom Macroglobulinemia]] | ||
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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 [[ | *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 ([[ | *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" /> | ||
*Bone marrow involvement is more common in patients with IgM-associated LPL ([[ | *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== | ||