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HAEM5:Splenic diffuse red pulp small B-cell lymphoma (view source)
Revision as of 14:40, 13 December 2023
, 14:40, 13 December 2023no edit summary
{{Under Construction}}
{{Under Construction}}
<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:Splenic Diffuse Red Pulp Small B-cell Lymphoma]].
<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:Splenic Diffuse Red Pulp Small B-cell Lymphoma]].
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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>
==Primary Author(s)*==
==Primary Author(s)*==
__TOC__
__TOC__
==Cancer Category/Type==
==Cancer Category / Type==
*[[Mature B-Cell Neoplasms|Mature B-Cell Neoplasm]]
*[[HAEM4:Mature B-Cell Neoplasms|Mature B-Cell Neoplasm]]
==Cancer Sub-Classification / Subtype==
==Cancer Sub-Classification / Subtype==
*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
==Definition / Description of Disease==
==Definition / Description of Disease==
|BRAF p.Val600Glu
|BRAF p.Val600Glu
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Hairy Cell Leukemia|HCL]] but rare/absent in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Hairy cell leukaemia|HCL]] but rare/absent in SDRPL<ref name=":3" />
|-
|-
|MAP2K1
|MAP2K1
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Hairy Cell Leukemia Variant|HCL-v]] and a subset of cases classified as [[Hairy Cell Leukemia|HCL]] but uncommon in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Hairy cell leukaemia Variant|HCL-v]] and a subset of cases classified as [[HAEM5:Hairy cell leukaemia|HCL]] but uncommon in SDRPL<ref name=":3" />
|-
|-
|KLF2 and TNFAIP3
|KLF2 and TNFAIP3
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Splenic Marginal Zone Lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Splenic marginal zone lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|-
|-
|MYD88
|MYD88
|Possible role in diagnosis (exclusion)
|Possible role in diagnosis (exclusion)
|Prevalent in [[Lymphoplasmacytic Lymphoma|LPL]] and [[Splenic Marginal Zone Lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|Prevalent in [[HAEM5:Lymphoplasmacytic lymphoma|LPL]] and [[HAEM5:Splenic marginal zone lymphoma|SMZL]] but rare/absent in SDRPL<ref name=":3" />
|-
|-
|BCOR
|BCOR
|Possible role in diagnosis (inclusion)
|Possible role in diagnosis (inclusion)
|Prevalent in SDRPL but rare/absent in [[Hairy Cell Leukemia|HCL]], [[Hairy Cell Leukemia Variant|HCL-v]], and [[Splenic Marginal Zone Lymphoma|SMZL]]<ref name=":3" />
|Prevalent in SDRPL but rare/absent in [[HAEM5:Hairy cell leukaemia|HCL]], [[HAEM5:Hairy cell leukaemia Variant|HCL-v]], and [[HAEM5:Splenic marginal zone lymphoma|SMZL]]<ref name=":3" />
|}
|}
</blockquote>
</blockquote>
==Individual Region Genomic Gain/Loss/LOH==
==Individual Region Genomic Gain / Loss / LOH==
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene fusions. Can include references in the table. Can refer to CGC workgroup tables as linked on the homepage if applicable.'') </span>
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene fusions. Can include references in the table. Can refer to CGC workgroup tables as linked on the homepage if applicable.'') </span>
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</blockquote>
==Gene Mutations (SNV/INDEL)==
==Gene Mutations (SNV / INDEL)==
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent and common as well either disease defining and/or clinically significant. Can include references in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity.'') </span>
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent and common as well either disease defining and/or clinically significant. Can include references in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity.'') </span>
*SDRPL is a provisional WHO entity and definitive diagnostic criteria have not been determined
*SDRPL is a provisional WHO entity and definitive diagnostic criteria have not been determined
**Diagnosis of exclusion
**Diagnosis of exclusion
***Differential of splenic lymphomas with cytoplasmic projections: [[Hairy Cell Leukemia|HCL]], [[Hairy Cell Leukemia Variant|HCL-v]], [[Splenic Marginal Zone Lymphoma|SMZL]]
***Differential of splenic lymphomas with cytoplasmic projections: [[HAEM5:Hairy cell leukaemia|HCL]], [[HAEM5:Hairy cell leukaemia Variant|HCL-v]], [[HAEM5:Splenic marginal zone lymphoma|SMZL]]
**Distinction is by clinical history, morphology, and immunohistochemistry
**Distinction is by clinical history, morphology, and immunohistochemistry
***No pathognomonic diagnostic markers (molecular or otherwise)
***No pathognomonic diagnostic markers (molecular or otherwise)
==Links==
==Links==
*[[Hairy Cell Leukemia]]
*[[HAEM5:Hairy cell leukaemia]]
*[[Hairy Cell Leukemia Variant]]
*[[HAEM5:Hairy cell leukaemia Variant]]
*[[Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[HAEM4:Splenic B-cell Lymphoma/Leukemia, Unclassifiable]]
*[[Splenic Marginal Zone Lymphoma]]
*[[HAEM5:Splenic marginal zone lymphoma]]
==References==
==References==