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{{DISPLAYTITLE:Early T-precursor lymphoblastic leukaemia / lymphoma}}
 
{{DISPLAYTITLE:Early T-precursor lymphoblastic leukaemia / lymphoma}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (5th ed.)]]
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[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]
    
{{Under Construction}}
 
{{Under Construction}}
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<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:Early T-Cell Precursor Lymphoblastic Leukemia]].
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<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:Early T-Cell Precursor Lymphoblastic Leukemia]].
 
}}</blockquote>
 
}}</blockquote>
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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); to add (or move) a row or column to a table, click within the table and select the > symbol that appears to be given options. 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>
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==Primary Author(s)*==
 
==Primary Author(s)*==
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__TOC__
 
__TOC__
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==Cancer Category/Type==
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==WHO Classification of Disease==
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[https://ccga.io/index.php/T-Lymphoblastic_Leukemia/Lymphoma T-Lymphoblastic Leukemia (T-ALL)]
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{| class="wikitable"
 
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!Structure
==Cancer Sub-Classification / Subtype==
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!Disease
 
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|-
Early T-Cell Precursor Lymphoblastic Leukemia (ETP-ALL)
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|Book
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|Haematolymphoid Tumours (5th ed.)
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|-
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|Category
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|T-cell and NK-cell lymphoid proliferations and lymphomas
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|-
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|Family
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|Precursor T-cell neoplasms
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|-
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|Type
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|T-lymphoblastic leukaemia / lymphoma
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|-
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|Subtype(s)
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|Early T-precursor lymphoblastic leukaemia / lymphoma
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|}
    
==Definition / Description of Disease==
 
==Definition / Description of Disease==
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==Clinical Features==
 
==Clinical Features==
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Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table'') </span>
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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"
 
{| class="wikitable"
 
|'''Signs and Symptoms'''
 
|'''Signs and Symptoms'''
|EXAMPLE Asymptomatic (incidental finding on complete blood counts)
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|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
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EXAMPLE B-symptoms (weight loss, fever, night sweats)
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<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
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EXAMPLE Fatigue
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<span class="blue-text">EXAMPLE:</span> Fatigue
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EXAMPLE Lymphadenopathy (uncommon)
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<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
 
|-
 
|-
 
|'''Laboratory Findings'''
 
|'''Laboratory Findings'''
|EXAMPLE Cytopenias
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|<span class="blue-text">EXAMPLE:</span> Cytopenias
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EXAMPLE Lymphocytosis (low level)
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<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
 
|}
 
|}
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!Notes
 
!Notes
 
|-
 
|-
|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 20% (COSMIC)
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|<span class="blue-text">EXAMPLE:</span> t(9;22)(q34;q11.2)||<span class="blue-text">EXAMPLE:</span> 3'ABL1 / 5'BCR||<span class="blue-text">EXAMPLE:</span> der(22)||<span class="blue-text">EXAMPLE:</span> 20% (COSMIC)
EXAMPLE 30% (add reference)
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<span class="blue-text">EXAMPLE:</span> 30% (add reference)
 
|Yes
 
|Yes
 
|No
 
|No
 
|Yes
 
|Yes
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).
 
The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).
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Other chromosomal rearrangements involving KMT2A have been observed in ETP-ALL <ref name=":6" />.  
 
Other chromosomal rearrangements involving KMT2A have been observed in ETP-ALL <ref name=":6" />.  
 
</blockquote>
 
</blockquote>
==Individual Region Genomic Gain/Loss/LOH==
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==Individual Region Genomic Gain / Loss / LOH==
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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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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. Do not delete table.'') </span>
    
{| class="wikitable sortable"
 
{| class="wikitable sortable"
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!Notes
 
!Notes
 
|-
 
|-
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
7
 
7
|EXAMPLE Loss
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|<span class="blue-text">EXAMPLE:</span> Loss
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
chr7:1- 159,335,973 [hg38]
 
chr7:1- 159,335,973 [hg38]
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
chr7
 
chr7
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|Yes
 
|Yes
 
|No
 
|No
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).
 
Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).
 
|-
 
|-
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
8
 
8
|EXAMPLE Gain
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|<span class="blue-text">EXAMPLE:</span> Gain
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
chr8:1-145,138,636 [hg38]
 
chr8:1-145,138,636 [hg38]
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
chr8
 
chr8
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|No
 
|No
 
|No
 
|No
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
Common recurrent secondary finding for t(8;21) (add reference).
 
Common recurrent secondary finding for t(8;21) (add reference).
 
|}
 
|}
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<blockquote class='blockedit'>{{Box-round|title=v4:Individual Region Genomic Gain/Loss/LOH|The content below was from the previous version of the page. Please incorporate above.}}
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<blockquote class='blockedit'>{{Box-round|title=v4:Individual Region Genomic Gain / Loss / LOH|The content below was from the previous version of the page. Please incorporate above.}}
    
Currently there is no specific copy number alterations/LOH that is associated with ETP-ALL.
 
Currently there is no specific copy number alterations/LOH that is associated with ETP-ALL.
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==Characteristic Chromosomal Patterns==
 
==Characteristic Chromosomal Patterns==
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Put your text here <span style="color:#0070C0">(''EXAMPLE PATTERNS: hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis'')</span>
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Put your text here <span style="color:#0070C0">(''EXAMPLE PATTERNS: hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis. Do not delete table.'')</span>
    
{| class="wikitable sortable"
 
{| class="wikitable sortable"
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!Notes
 
!Notes
 
|-
 
|-
|EXAMPLE
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|<span class="blue-text">EXAMPLE:</span>
    
Co-deletion of 1p and 18q
 
Co-deletion of 1p and 18q
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|No
 
|No
 
|No
 
|No
|EXAMPLE:
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|<span class="blue-text">EXAMPLE:</span>
    
See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
 
See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
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Currently there is no specific chromosomal alteration that is characteristic for ETP-ALL.
 
Currently there is no specific chromosomal alteration that is characteristic for ETP-ALL.
 
</blockquote>
 
</blockquote>
==Gene Mutations (SNV/INDEL)==
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==Gene Mutations (SNV / INDEL)==
    
Genes encoding transcription factors for development and differentiation (''ETV6, GATA3, HOXA, LMO2, RUNX1, WT1''), kinase signaling (''FLT3, JAK1, JAK3, IL7R, KRAS, NRAS''), and epigenetic modifiers (''DNMT3A, EED, EZH2, PHF6, SUZ12'') are commonly mutated in ETP-ALL <ref name=":5" />. More typical T-ALL mutations, such as ''NOTCH1'' mutations and CDKN1/2 mutations are less frequent in ETP-ALL <ref name=":2" />.
 
Genes encoding transcription factors for development and differentiation (''ETV6, GATA3, HOXA, LMO2, RUNX1, WT1''), kinase signaling (''FLT3, JAK1, JAK3, IL7R, KRAS, NRAS''), and epigenetic modifiers (''DNMT3A, EED, EZH2, PHF6, SUZ12'') are commonly mutated in ETP-ALL <ref name=":5" />. More typical T-ALL mutations, such as ''NOTCH1'' mutations and CDKN1/2 mutations are less frequent in ETP-ALL <ref name=":2" />.
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<nowiki>*</nowiki>''Citation of this Page'': “Early T-precursor lymphoblastic leukaemia / lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Early_T-precursor_lymphoblastic_leukaemia_/_lymphoma</nowiki>.[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases E]]
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<nowiki>*</nowiki>''Citation of this Page'': “Early T-precursor lymphoblastic leukaemia / lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Early_T-precursor_lymphoblastic_leukaemia_/_lymphoma</nowiki>.
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[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases E]]