HAEM5:Myelodysplastic/myeloproliferative neoplasm, NOS: Difference between revisions

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*Overlap with [[HAEM4:Myelodysplastic Syndromes (MDS)|MDS]] and [[HAEM4:Myeloproliferative Neoplasms (MPN)|MPN]] <ref name=":0" />
*Overlap with [[HAEM4:Myelodysplastic Syndromes (MDS)|MDS]] and [[HAEM4:Myeloproliferative Neoplasms (MPN)|MPN]] <ref name=":0" />
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*Does not have features that define it as belonging to any of the other categories of MDS/MPN <ref name=":0" /><ref name=":1" />
*Does not have features that define it as belonging to any of the other categories of MDS/MPN <ref name=":0" /><ref name=":1" />


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==Sites of Involvement==
==Sites of Involvement==
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May be similar to that of [[HAEM4:Myelodysplastic Syndromes (MDS)|MDS]] or [[HAEM4:Myeloproliferative Neoplasms (MPN)|MPN]] <ref name=":0" />
May be similar to that of [[HAEM4:Myelodysplastic Syndromes (MDS)|MDS]] or [[HAEM4:Myeloproliferative Neoplasms (MPN)|MPN]] <ref name=":0" />


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==Chromosomal Rearrangements (Gene Fusions)==
==Chromosomal Rearrangements (Gene Fusions)==
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*None identified
*None identified
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*Absence of ''PDGFRA'', ''PDGFRB'' and ''FGFR1'' rearrangements, absence of ''PCM1-JAK2'' fusion <ref name=":0" />
*Absence of ''PDGFRA'', ''PDGFRB'' and ''FGFR1'' rearrangements, absence of ''PCM1-JAK2'' fusion <ref name=":0" />


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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>


*Prognosis is variable and not well documented <ref name=":0" />
*Prognosis is variable and not well documented <ref name=":0" />
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*“Treatment is based on therapies used for MDS or MPN and is guided by symptoms and/or cytopenias” and can include growth factors for leukocytosis or cytoreductive therapies for cytopenias <ref name=":0" />.
*“Treatment is based on therapies used for MDS or MPN and is guided by symptoms and/or cytopenias” and can include growth factors for leukocytosis or cytoreductive therapies for cytopenias <ref name=":0" />.


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==Individual Region Genomic Gain / Loss / LOH==
==Individual Region Genomic Gain / Loss / LOH==
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One study identified very few recurrent gains and losses below the level of cytogenetic detection <ref name=":3" />.  
One study identified very few recurrent gains and losses below the level of cytogenetic detection <ref name=":3" />.  


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==Characteristic Chromosomal Patterns==
==Characteristic Chromosomal Patterns==
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*None specific to MDS/MPN-U
*None specific to MDS/MPN-U
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*Excludes any case with isolated del(5q) (even if ''JAK2'' is mutated) <ref name=":0" /> - these should be classified as MDS with isolated del(5q).
*Excludes any case with isolated del(5q) (even if ''JAK2'' is mutated) <ref name=":0" /> - these should be classified as MDS with isolated del(5q).


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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>


Amongst the MDS/MPN subtypes, MDS/MPN-U has the most heterogeneous mutation profile. In one study it was most strongly correlated with ''U2AF1'' and ''TP53'' mutations <ref name=":3" />.
Amongst the MDS/MPN subtypes, MDS/MPN-U has the most heterogeneous mutation profile. In one study it was most strongly correlated with ''U2AF1'' and ''TP53'' mutations <ref name=":3" />.
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There is a significant likelihood of AML developing if there is ''ASXL1'' and ''SRSF2'' co-mutation <ref name=":2" />.
There is a significant likelihood of AML developing if there is ''ASXL1'' and ''SRSF2'' co-mutation <ref name=":2" />.


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==Epigenomic Alterations==
==Epigenomic Alterations==
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*Major drivers / early mutations in epigenetic regulators (''ASXL1, TET2)'' and splicing factors (''SRSF2'') <ref name=":2" /> <ref name=":3" />
*Major drivers / early mutations in epigenetic regulators (''ASXL1, TET2)'' and splicing factors (''SRSF2'') <ref name=":2" /> <ref name=":3" />
*Secondary mutations in signaling pathway genes (''JAK2'') <ref name=":3" />
*Secondary mutations in signaling pathway genes (''JAK2'') <ref name=":3" />


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