Compendium of Cancer Genome Aberrations

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{{DISPLAYTITLE:Lymphoplasmacytic lymphoma}}
{{DISPLAYTITLE:Lymphoplasmacytic lymphoma}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]


{{Under Construction}}
{{Under Construction}}


<blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Lymphoplasmacytic Lymphoma]].
<blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Lymphoplasmacytic Lymphoma]].
Other relevent pages include: [[HAEM4:Waldenstrom Macroglobulinemia]]
Other relevent pages include: [[HAEM4:Waldenstrom Macroglobulinemia]]


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==Definition / Description of Disease==
*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 [[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>
==Synonyms / Terminology==
Malignant lymphoma, lymphoplasmacytoid lymphoma.<ref name=":0" />
==Epidemiology / Prevalence==
*Lymphoplasmacytic lymphoma, in approximately 95% of cases, occurs in patients of Caucasian descent. <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.
*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==
{| class="wikitable"
|'''Signs and Symptoms'''
|Weakness (usually related to anemia)
Fatigue
Hyperviscosity (in 30% of cases)
Lymphadenopathy and splenomegaly (in 10-20% of patient at time of diagnosis)
Paraprotein deposition in tissues with organ-specific dysfunction:
*Neuropathy (minority of patients; may be related to the IgM paraprotein with myelin sheath antigens)
*Diarrhea (rare, due to paraprotein deposition in gastrointestinal tract)
*Coagulopathy (may be cause by IgM binding to clotting factors)
*Bullae or papules in the skin
*Proteinuria and renal failure
|-
|'''Laboratory Findings'''
|IgM paraprotein
IgM and IgG paraprotein (minority of patients)
Cold agglutinins
Cryoglobulinemia
|}
<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.}}</blockquote><ref name=":0" /><ref name=":1" /><blockquote class="blockedit">
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==Sites of Involvement==
*Bone marrow (most of cases), lymph nodes, spleen, liver, and other extranodal sites. The peripheral blood may also be involved.
*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" />
*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==
*Bone marrow involvement is characterized by diffuse, interstitial, and nodular infiltration patterns. Paratrabecular can also be  present; however, it has  been observed more frequently in cases of [[marginal zone lymphoma.]]
*The infiltrate is characterized by small monotonous lymphocytes admixed with a variable amount of plasmacytoid lymphocytes and plasma cells. Although nonspecific, variable amount of reactive mast cell and hemosiderin deposits are frequently observed. Dutcher bodies (nuclear vacuoles containing IgM protein) and Russell bodies (cytoplasmic inclusion) are often seen similarly in multiple myeloma cases. <ref name=":0" />
*Peripheral blood often demonstrates lymphoma cells with similar morphology. Leukocytosis is not typically observed. Peripheral blood may also demonstrate a rouleaux formation due to increased IgM paraprotein.
==Immunophenotype==
Put your text here and fill in the table
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (subset)
|IgM
|-
|Positive (subset)
|IgG
|-
|Positive (subset)
|IgA
|-
|Positive (universal)||CD19
|-
|Positive (universal)||CD20
|-
|Positive (universal)||CD22 (dim)
|-
|Positive (universal)||CD25
|-
|Positive (universal)
|CD79a
|-
|Positive (universal)
|CD45
|-
|Positive (variable)
|CD38
|-
|Positive (universal)
|PAX5
|-
|Positive (subset)
|CD5
|-
|Positive (subset)
|CD10
|-
|Positive (subset)
|TCL1
|-
|Negative (universal)
|Cyclin D1
|-
|Negative (universal)
|LEF1
|-
|Negative (universal)
|EBV stain
|-
|Negative (universal)
|CD56
|-
|Negative (universal)
|CD117
|}
<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.}}</blockquote><ref name=":1" /><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
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==WHO Essential and Desirable Genetic Diagnostic Criteria==
==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>
<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>
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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.}}</blockquote><ref name=":0" /><ref name=":1" /><blockquote class="blockedit">
<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.}}</blockquote><ref name=":0" /><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><blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
<center><span style="color:Maroon">'''End of V4 Section'''</span>
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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>.
<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]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases L]]
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