HAEM5:Diffuse large B-cell lymphoma, NOS: Difference between revisions

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{{DISPLAYTITLE:Diffuse large B-cell lymphoma, NOS}}
{{DISPLAYTITLE:Diffuse large B-cell lymphoma, NOS}}
[[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:Diffuse Large B-cell Lymphoma, Not Otherwise Specified]].
<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:Diffuse Large B-cell Lymphoma, Not Otherwise Specified]].
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|Subtype(s)
|Subtype(s)
|Diffuse large B-cell lymphoma, NOS
|Diffuse large B-cell lymphoma, NOS
|}
==Definition / Description of Disease==
Non-Hodgkin lymphoma (NHL) is one of the most frequently diagnosed cancer types representing approximately 4% of cancers Worldwide. The most common type of NHL is Diffuse Large B-Cell Lymphoma, Not Otherwise Specified (DLBCL, NOS) accounting for 30% of all NHL cases.
==Synonyms / Terminology==
DLBCL, NOS
==Epidemiology / Prevalence==
DLBCL, NOS constitutes close to 30% of NHL. It is more common in the elderly with an average age of 60 years, but it is also seen in all age groups. Put your text here
==Clinical Features==
Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
<span class="blue-text">EXAMPLE:</span> Fatigue
<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias
<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}
<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>
The primary site of lymphoma, either the lymph node or extra nodal site, is important in assessing clinical features, treatment options and outcome of the disease. Clinical presentation of DLBCL can be variable, usually depends on the site of disease involvement, rapid growth rate, enlarged lymph nodes, extra nodal mass infiltrating into tissues or obstructing organs. The enlarged lymphomatous mass may also exert severe pain. Patients usually experience fever, drenching night sweats, weight loss, anorexia, pedal edema (due to extensive pelvic lymphadenopathy), fatigue, chest discomfort or shortness of breath due to mediastinal lymphadenopathy. Put your text here
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<center><span style="color:Maroon">'''End of V4 Section'''</span>
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==Sites of Involvement==
Lymph node or extra nodal site, enlarged lymph nodes, extra nodal mass infiltrating into tissues or obstructing organs. The enlarged lymphomatous mass may also exert severe pain.
==Morphologic Features==
Clinical presentation of DLBCL can be variable, usually depends on the site of disease involvement, rapid growth rate, enlarged lymph nodes, extra nodal mass infiltrating into tissues or obstructing organs. The enlarged lymphoma mass may also exert severe pain. Patients usually experience fever, drenching night sweats, weight loss, anorexia, pedal edema (due to extensive pelvic lymphadenopathy), fatigue, chest discomfort or shortness of breath due to mediastinal lymphadenopathy.
==Immunophenotype==
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{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||<span class="blue-text">EXAMPLE:</span> CD1
|-
|Positive (subset)||<span class="blue-text">EXAMPLE:</span> CD2
|-
|Negative (universal)||<span class="blue-text">EXAMPLE:</span> CD3
|-
|Negative (subset)||<span class="blue-text">EXAMPLE:</span> CD4
|}
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<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
<blockquote class="blockedit">{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
* Chromosomal Rearrangements (Gene Fusions)
* Chromosomal Rearrangements (Gene Fusions)
* Individual Region Genomic Gain/Loss/LOH
* Individual Region Genomic Gain/Loss/LOH
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'''AMPLIFICATION''': BCL2, REL, CD274, PDCD1LG2, JAK2, KRAs, TBL1XR1, RB1
'''AMPLIFICATION''': BCL2, REL, CD274, PDCD1LG2, JAK2, KRAs, TBL1XR1, RB1
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<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}</blockquote>
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Due to the clinicopathologic, biological and genetic diversity, DLBCL is sub divided into morphological variants and molecular subtypes. Initial workup and evaluation of DLBCL has become increasingly complex partly due to the genetic abnormalities that are targets for specific therapy and play an important role in monitoring residual disease. Genomic studies help in clinical management, risk stratification, enrolling patients onto treatment protocols, clinical trials, and detection of therapeutic targets. GS, If IHC is positive for GCB like DLBCL, FISH and Cytogenetics for MYC, BCL2 or BCL6 gene rearrangement are recommended to rule out double and triple hit lymphoma.  
Due to the clinicopathologic, biological and genetic diversity, DLBCL is sub divided into morphological variants and molecular subtypes. Initial workup and evaluation of DLBCL has become increasingly complex partly due to the genetic abnormalities that are targets for specific therapy and play an important role in monitoring residual disease. Genomic studies help in clinical management, risk stratification, enrolling patients onto treatment protocols, clinical trials, and detection of therapeutic targets. GS, If IHC is positive for GCB like DLBCL, FISH and Cytogenetics for MYC, BCL2 or BCL6 gene rearrangement are recommended to rule out double and triple hit lymphoma.  
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|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


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'''SNV''' : MLL2, TP53, MYD88, B2M, CREBBP, TNFAIP3, PIM1, BCL2, EZH2, TNFRSF14, CD79B
'''SNV''' : MLL2, TP53, MYD88, B2M, CREBBP, TNFAIP3, PIM1, BCL2, EZH2, TNFRSF14, CD79B
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<blockquote class='blockedit'>{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}</blockquote>
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B-Cell differentiation, TP53 pathway, NF-kB pathway, Apoptosis, Cell migration, Immune response,BCR-MYD88 signaling, PI3K-AKT-mTCR pathway
B-Cell differentiation, TP53 pathway, NF-kB pathway, Apoptosis, Cell migration, Immune response,BCR-MYD88 signaling, PI3K-AKT-mTCR pathway
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(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking where you want to insert the reference, selecting the “Cite” icon at the top of the wiki page, and using the “Automatic” tab option to search by PMID to select the reference to insert. If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference. To insert the same reference again later in the page, select the “Cite” icon and “Re-use” to find the reference; DO NOT insert the same reference twice using the “Automatic” tab as it will be treated as two separate references. The reference list in this section will be automatically generated and sorted''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">)</span> <references />
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'''
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==Notes==
==Notes==
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<nowiki>*</nowiki>''Citation of this Page'': “Diffuse large B-cell lymphoma, NOS”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Diffuse_large_B-cell_lymphoma,_NOS</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Diffuse large B-cell lymphoma, NOS”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Diffuse_large_B-cell_lymphoma,_NOS</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases D]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases D]]