HAEM5:ALK-negative anaplastic large cell lymphoma: Difference between revisions
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==Related Terminology== | ==Related Terminology== | ||
{| class="wikitable" | {| class="wikitable" | ||
|+ | |+ | ||
|Acceptable | |Acceptable | ||
| | |Anaplastic large cell lymphoma, ALK-negative | ||
|- | |- | ||
|Not Recommended | |Not Recommended | ||
| | |N/A | ||
|} | |} | ||
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*<nowiki>*</nowiki> These rearrangements are considered mutually exclusive; however, a single case with both ''DUSP22'' and ''TP63'' rearrangement has been described<ref name=":11" />. Can also be seen in a fraction of other PTCL. | *<nowiki>*</nowiki> These rearrangements are considered mutually exclusive; however, a single case with both ''DUSP22'' and ''TP63'' rearrangement has been described<ref name=":11" />. Can also be seen in a fraction of other PTCL. | ||
*5-year overall survival > 90% | *5-year overall survival > 90% | ||
* | *Therapeutic Implications | ||
**Multi-agent chemotherapy (CHOEP or CHOP-based) as first-line, with or without radiotherapy of involved site | **Multi-agent chemotherapy (CHOEP or CHOP-based) as first-line, with or without radiotherapy of involved site | ||
**High dose chemotherapy and autologous stem cell transplantation for remission | **High dose chemotherapy and autologous stem cell transplantation for remission | ||
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{| class="wikitable sortable" | {| class="wikitable sortable" | ||
|- | |- | ||
!Chr #!! | !Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s) | ||
! | !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | ||
! | !Established Clinical Significance Per Guidelines - Yes or No (Source) | ||
! | !Clinical Relevance Details/Other Notes | ||
|- | |- | ||
|<span class="blue-text">EXAMPLE:</span> | |<span class="blue-text">EXAMPLE:</span> | ||
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*Prevalence 19-26% | *Prevalence 19-26% | ||
|- | |- | ||
| | |6q | ||
| | |Loss > CN-LOH | ||
|21 | |21 | ||
| | | | ||
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| | | | ||
*Prevalence 35% | *Prevalence 35% | ||
*Genes affected: | *Genes affected: ''PRDM1, ATG5'' | ||
|- | |- | ||
|10p | |10p | ||
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|17p | |17p | ||
|Loss | |Loss | ||
| | |13.3-p12 | ||
| | | | ||
|No | |No | ||
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19% | 19% | ||
|- | |- | ||
| | |6q | ||
| | |Loss > CN-LOH; | ||
See also below for somatic mutations | See also below for somatic mutations | ||
| | |21 | ||
| | |PRDM1, ATG5 | ||
| | |35% | ||
|- | |- | ||
|10p | |10p | ||
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|29% | |29% | ||
|- | |- | ||
| | |17p | ||
| | |Loss | ||
| | |13.3-p12 | ||
| | |TP53 | ||
| | |42% | ||
|} | |} | ||
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!Chromosomal Pattern | !Chromosomal Pattern | ||
!Molecular Pathogenesis | !Molecular Pathogenesis | ||
! | !Prevalence - | ||
Common >20%, Recurrent 5-20% or Rare <5% (Disease) | |||
! | !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | ||
! | !Established Clinical Significance Per Guidelines - Yes or No (Source) | ||
! | !Clinical Relevance Details/Other Notes | ||
|- | |- | ||
|<span class="blue-text">EXAMPLE:</span> | |<span class="blue-text">EXAMPLE:</span> | ||
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{| class="wikitable sortable" | {| class="wikitable sortable" | ||
|- | |- | ||
!Gene!! | !Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence - | ||
Common >20%, Recurrent 5-20% or Rare <5% (Disease) | |||
! | !Diagnostic, Prognostic, and Therapeutic Significance - D, P, T | ||
! | !Established Clinical Significance Per Guidelines - Yes or No (Source) | ||
! | !Clinical Relevance Details/Other Notes | ||
|- | |- | ||
|<span class="blue-text">EXAMPLE:</span>''EGFR'' | |<span class="blue-text">EXAMPLE:</span>''EGFR'' | ||
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{| class="wikitable sortable" | {| class="wikitable sortable" | ||
|- | |- | ||
!Gene; Genetic Alteration!! | !Gene; Genetic Alteration!!Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)!!Prevalence (COSMIC / TCGA / Other)!!Concomitant Mutations!!Mutually Exclusive Mutations | ||
! | !Diagnostic Significance (Yes, No or Unknown) | ||
!Prognostic Significance (Yes, No or Unknown) | !Prognostic Significance (Yes, No or Unknown) | ||
!Therapeutic Significance (Yes, No or Unknown) | !Therapeutic Significance (Yes, No or Unknown) | ||
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|Positive (universal)||CD30*, CD43 (almost universally) | |Positive (universal)||CD30*, CD43 (almost universally) | ||
|- | |- | ||
|Negative (universal)|| | |Negative (universal)||ALK, TP63, EBER, LMP-1 | ||
|- | |- | ||
|Positive (frequent) | |Positive (frequent) | ||
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!Finding!!Marker | !Finding!!Marker | ||
|- | |- | ||
|Positive (universal)||CD30*, CD43 (almost universally), | |Positive (universal)||CD30*, CD43 (almost universally), P63, CD4+ cases more common than CD8 | ||
|- | |- | ||
|Negative (universal)|| | |Negative (universal)||ALK, EBER, LMP-1 | ||
|- | |- | ||
|Positive (frequent) | |Positive (frequent) | ||
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|Positive (universal)||CD30*, CD43 (almost universally), CD2, CD3, CD4+ cases more common than CD8, CD5, TIA1, granzyme B, perforin, EMA | |Positive (universal)||CD30*, CD43 (almost universally), CD2, CD3, CD4+ cases more common than CD8, CD5, TIA1, granzyme B, perforin, EMA | ||
|- | |- | ||
|Negative (universal)|| | |Negative (universal)||ALK, P63, EBER, LMP-1 | ||
|- | |- | ||
|Positive (common) | |Positive (common) | ||