HAEM5:Mycosis fungoides: Difference between revisions

*Pruritic, erythematous or poikilodermatous and atrophic skin lesions with a fine scale

*May have symptoms and/or diagnosis of other inflammatory skin diseases (e.g., eczema or psoriasis) for 3 - 4 years prior to the diagnosis of MF<ref>{{Cite journal|last=Kim|first=Youn H.|last2=Liu|first2=Howard L.|last3=Mraz-Gernhard|first3=Serena|last4=Varghese|first4=Anna|last5=Hoppe|first5=Richard T.|date=2003-07-01|title=Long-term Outcome of 525 Patients With Mycosis Fungoides and Sézary Syndrome: Clinical Prognostic Factors and Risk for Disease Progression|url=http://archderm.jamanetwork.com/article.aspx?doi=10.1001/archderm.139.7.857|journal=Archives of Dermatology|language=en|volume=139|issue=7|doi=10.1001/archderm.139.7.857|issn=0003-987X}}</ref>

</blockquote>

==Sites of Involvement==

*No consistent gene fusions

*''CD28-CTLA4'' gene fusion identified, resulting in activation of TCR signaling <ref>{{Cite journal|last=Ungewickell|first=Alexander|last2=Bhaduri|first2=Aparna|last3=Rios|first3=Eon|last4=Reuter|first4=Jason|last5=Lee|first5=Carolyn S|last6=Mah|first6=Angela|last7=Zehnder|first7=Ashley|last8=Ohgami|first8=Robert|last9=Kulkarni|first9=Shashikant|date=2015-09|title=Genomic analysis of mycosis fungoides and Sézary syndrome identifies recurrent alterations in TNFR2|url=http://www.nature.com/articles/ng.3370|journal=Nature Genetics|language=en|volume=47|issue=9|pages=1056–1060|doi=10.1038/ng.3370|issn=1061-4036|pmc=PMC6091217|pmid=26258847}}</ref>

</blockquote>

* Individual Region Genomic Gain/Loss/LOH

* Characteristic Chromosomal Patterns

The staging system for mycosis fungoides, which also includes Sézary syndrome, was updated in 2007 by the International Society for Cutaneous Lymphomas (ISCL) and the European Organization of Research and Treatment of Cancer (EORTC) <ref>{{Cite journal|last=Olsen|first=Elise|last2=Vonderheid|first2=Eric|last3=Pimpinelli|first3=Nicola|last4=Willemze|first4=Rein|last5=Kim|first5=Youn|last6=Knobler|first6=Robert|last7=Zackheim|first7=Herschel|last8=Duvic|first8=Madeleine|last9=Estrach|first9=Teresa|date=2007-09-15|title=Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)|url=https://pubmed.ncbi.nlm.nih.gov/17540844|journal=Blood|volume=110|issue=6|pages=1713–1722|doi=10.1182/blood-2007-03-055749|issn=0006-4971|pmid=17540844}}</ref>.  The revised TNMB staging of MF/SS determines management and treatment, and has been demonstrated to have prognostic significance. <ref name=":0" />  The TNMB staging forms the basis for a risk-adapted approach to treatment of mycosis fungoides.

{| class="wikitable"

|}

</blockquote>

==Individual Region Genomic Gain / Loss / LOH==

|}

Mycosis fungoides does not have a defining disease specific molecular abnormality.  However, the molecular profile of mycosis fungoides and other CTCLs continues to be investigated.   

</blockquote>

==Characteristic Chromosomal Patterns==

|}

Complex chromosomal abnormalities have been identified in mycosis fungoides, usually in tumor stage.  Specific translocations have not been identified.   

</blockquote>

==Gene Mutations (SNV / INDEL)==

Although no disease specific molecular abnormalities exist in mycosis fungoides, but some changes are seen more frequently than others.<ref name=":5">{{Cite journal|last=Walia|first=Ritika|last2=Yeung|first2=Cecilia C. S.|date=2020-01-22|title=An Update on Molecular Biology of Cutaneous T Cell Lymphoma|url=https://www.frontiersin.org/article/10.3389/fonc.2019.01558/full|journal=Frontiers in Oncology|volume=9|pages=1558|doi=10.3389/fonc.2019.01558|issn=2234-943X|pmc=PMC6987372|pmid=32039026}}</ref>  Single copy number variations (SCNV) are important mutational drivers for CTCLs and mycosis fungoides and are seen with greater frequency than somatic single nucleotide variations (SSNV) when compared to other cancers with significantly higher SCNV/SSNV ratios.<ref name=":6" /> <ref name=":3" />  Alterations in tumor suppressor genes are frequently implication the pathogenesis of mycosis fungoides, and more than 90% of variants arise from copy number alterations.  <ref name=":6" />   

|}

</blockquote>

==Epigenomic Alterations==

|}

</blockquote>

==Genetic Diagnostic Testing Methods==