HAEM5:Myelodysplastic/myeloproliferative neoplasm with neutrophilia: Difference between revisions

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<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}
<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}</blockquote>


The primary symptoms are related to splenomegaly and blood cell changes like anemia and/or thrombocytopenia<ref name=":1" /><ref name=":3">{{Cite journal|last=R|first=Kurzrock|last2=Ce|first2=Bueso-Ramos|last3=H|first3=Kantarjian|last4=E|first4=Freireich|last5=Sl|first5=Tucker|last6=M|first6=Siciliano|last7=S|first7=Pilat|last8=M|first8=Talpaz|date=2001|title=BCR Rearrangement-Negative Chronic Myelogenous Leukemia Revisited|url=https://pubmed.ncbi.nlm.nih.gov/11387365/|language=en|pmid=11387365}}</ref><ref name=":2" />.
The primary symptoms are related to splenomegaly and blood cell changes like anemia and/or thrombocytopenia<ref name=":1" /><ref name=":3">{{Cite journal|last=R|first=Kurzrock|last2=Ce|first2=Bueso-Ramos|last3=H|first3=Kantarjian|last4=E|first4=Freireich|last5=Sl|first5=Tucker|last6=M|first6=Siciliano|last7=S|first7=Pilat|last8=M|first8=Talpaz|date=2001|title=BCR Rearrangement-Negative Chronic Myelogenous Leukemia Revisited|url=https://pubmed.ncbi.nlm.nih.gov/11387365/|language=en|pmid=11387365}}</ref><ref name=":2" />.


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==Sites of Involvement==
==Sites of Involvement==
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No specific immunophenotypic characteristics (flow cytometric analysis of blood and bone marrow usually report as normal, except mildly increased myeloblasts in some cases).
No specific immunophenotypic characteristics (flow cytometric analysis of blood and bone marrow usually report as normal, except mildly increased myeloblasts in some cases).


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==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
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None.
None.
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* Individual Region Genomic Gain/Loss/LOH
* Individual Region Genomic Gain/Loss/LOH
* Characteristic Chromosomal Patterns
* Characteristic Chromosomal Patterns
* Gene Mutations (SNV/INDEL)}}
* Gene Mutations (SNV/INDEL)}}</blockquote>


Genetic abnormalities and mutations in related genes provide evidence of a clonal process to support a neoplastic process. However, they are not specific for aCML.  
Genetic abnormalities and mutations in related genes provide evidence of a clonal process to support a neoplastic process. However, they are not specific for aCML.  
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*Increased blasts in blood and/or bone marrow.
*Increased blasts in blood and/or bone marrow.


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==Individual Region Genomic Gain / Loss / LOH==
==Individual Region Genomic Gain / Loss / LOH==
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Karyotypic abnormalities are reported in as many as 80% of cases.  
Karyotypic abnormalities are reported in as many as 80% of cases.  
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*Isolated isochromosome 17q rare (most present with features of [[HAEM5:Chronic myelomonocytic leukaemia|CMML]] instead of aCML).
*Isolated isochromosome 17q rare (most present with features of [[HAEM5:Chronic myelomonocytic leukaemia|CMML]] instead of aCML).


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==Characteristic Chromosomal Patterns==
==Characteristic Chromosomal Patterns==
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None.
None.


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==Gene Mutations (SNV / INDEL)==
==Gene Mutations (SNV / INDEL)==
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<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}</blockquote>


{| class="wikitable sortable"
{| class="wikitable sortable"
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Mutations in ''TET2, ASXL1, EZH2'' and ''SRSF2'' (overlapping with [[HAEM5:Chronic myelomonocytic leukaemia|CMML]]) are not uncommon in aCML<ref>{{Cite journal|last=Faisal|first=Muhammad|last2=Stark|first2=Helge|last3=Büsche|first3=Guntram|last4=Schlue|first4=Jerome|last5=Teiken|first5=Kristin|last6=Kreipe|first6=Hans H.|last7=Lehmann|first7=Ulrich|last8=Bartels|first8=Stephan|date=2019|title=Comprehensive mutation profiling and mRNA expression analysis in atypical chronic myeloid leukemia in comparison with chronic myelomonocytic leukemia|url=http://doi.wiley.com/10.1002/cam4.1946|journal=Cancer Medicine|language=en|volume=8|issue=2|pages=742–750|doi=10.1002/cam4.1946|pmc=PMC6382710|pmid=30635983}}</ref>.
Mutations in ''TET2, ASXL1, EZH2'' and ''SRSF2'' (overlapping with [[HAEM5:Chronic myelomonocytic leukaemia|CMML]]) are not uncommon in aCML<ref>{{Cite journal|last=Faisal|first=Muhammad|last2=Stark|first2=Helge|last3=Büsche|first3=Guntram|last4=Schlue|first4=Jerome|last5=Teiken|first5=Kristin|last6=Kreipe|first6=Hans H.|last7=Lehmann|first7=Ulrich|last8=Bartels|first8=Stephan|date=2019|title=Comprehensive mutation profiling and mRNA expression analysis in atypical chronic myeloid leukemia in comparison with chronic myelomonocytic leukemia|url=http://doi.wiley.com/10.1002/cam4.1946|journal=Cancer Medicine|language=en|volume=8|issue=2|pages=742–750|doi=10.1002/cam4.1946|pmc=PMC6382710|pmid=30635983}}</ref>.


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==Epigenomic Alterations==
==Epigenomic Alterations==
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Frequently involved:  
Frequently involved:  
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*PI3K/AKT and MAPK/ERK (CSF3R)
*PI3K/AKT and MAPK/ERK (CSF3R)


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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==