Mycosis fungoides pathophysiology: Difference between revisions

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* [[Malignant]] [[T cell]] express [[adhesion]] [[Molecule|molecules]] such as [[CCR4]] and [[CLA]].<ref name="FossGirardi2017">{{cite journal|last1=Foss|first1=Francine M.|last2=Girardi|first2=Michael|title=Mycosis Fungoides and Sezary Syndrome|journal=Hematology/Oncology Clinics of North America|volume=31|issue=2|year=2017|pages=297–315|issn=08898588|doi=10.1016/j.hoc.2016.11.008}}</ref>
* [[Malignant]] [[T cell]] express [[adhesion]] [[Molecule|molecules]] such as [[CCR4]] and [[CLA]].<ref name="FossGirardi2017">{{cite journal|last1=Foss|first1=Francine M.|last2=Girardi|first2=Michael|title=Mycosis Fungoides and Sezary Syndrome|journal=Hematology/Oncology Clinics of North America|volume=31|issue=2|year=2017|pages=297–315|issn=08898588|doi=10.1016/j.hoc.2016.11.008}}</ref>
* [[Immunohistochemistry]] shows [[expression]] [[T cell|T-cell]] [[Antigen|antigens]] such as [[CD7]], [[CD5]], CD26, [[CD2]] and [[CD3 (immunology)|CD3]].<ref name="FossGirardi2017" />
* [[Immunohistochemistry]] shows [[expression]] [[T cell|T-cell]] [[Antigen|antigens]] such as [[CD7]], [[CD5]], CD26, [[CD2]] and [[CD3 (immunology)|CD3]].<ref name="FossGirardi2017" />
* Mycosis fungoides is T-cell lymphomas that primary manifest as multiple [[cutaneous]] [[Lesion|lesions]].<ref name="BagheraniSmoller2016">{{cite journal|last1=Bagherani|first1=Nooshin|last2=Smoller|first2=Bruce R.|title=An overview of cutaneous T cell lymphomas|journal=F1000Research|volume=5|year=2016|pages=1882|issn=2046-1402|doi=10.12688/f1000research.8829.1}}</ref>
* Mycosis fungoides is [[T cell|T-cell]] [[Lymphoma|lymphomas]] that primary manifest as multiple [[cutaneous]] [[Lesion|lesions]].<ref name="BagheraniSmoller2016">{{cite journal|last1=Bagherani|first1=Nooshin|last2=Smoller|first2=Bruce R.|title=An overview of cutaneous T cell lymphomas|journal=F1000Research|volume=5|year=2016|pages=1882|issn=2046-1402|doi=10.12688/f1000research.8829.1}}</ref>
* Mycosis Fungoides is the most common type of [[cutaneous T cell lymphoma]].<ref name="MahalingamReddy2015">{{cite journal|last1=Mahalingam|first1=Meera|last2=Reddy|first2=Vijaya B.|title=Mycosis Fungoides, Then and Now… Have We Travelled?|journal=Advances In Anatomic Pathology|volume=22|issue=6|year=2015|pages=376–383|issn=1072-4109|doi=10.1097/PAP.0000000000000092}}</ref>
* Mycosis Fungoides is the most common type of [[cutaneous T cell lymphoma]].<ref name="MahalingamReddy2015">{{cite journal|last1=Mahalingam|first1=Meera|last2=Reddy|first2=Vijaya B.|title=Mycosis Fungoides, Then and Now… Have We Travelled?|journal=Advances In Anatomic Pathology|volume=22|issue=6|year=2015|pages=376–383|issn=1072-4109|doi=10.1097/PAP.0000000000000092}}</ref>


==Microscopic Pathology==
==Microscopic Pathology==
* Mycosis fungoides pathological course may be divided into three main stages:<ref name="SongWillemze2013">{{cite journal|last1=Song|first1=Sophie X.|last2=Willemze|first2=Rein|last3=Swerdlow|first3=Steven H.|last4=Kinney|first4=Marsha C.|last5=Said|first5=Jonathan W.|title=Mycosis Fungoides|journal=American Journal of Clinical Pathology|volume=139|issue=4|year=2013|pages=466–490|issn=1943-7722|doi=10.1309/AJCPOBDP2OQAJ5BR}}</ref><ref name="VoraAnjaneyan2012">{{cite journal|last1=Vora|first1=Rita|last2=Anjaneyan|first2=Gopikrishnan|last3=Mubashir|first3=Syed|last4=Talavia|first4=Parag|title=Mycosis Fungoides: Tumour d′emblee|journal=Indian Dermatology Online Journal|volume=3|issue=2|year=2012|pages=122|issn=2229-5178|doi=10.4103/2229-5178.96709}}</ref><ref name="Tirumalae2013">{{cite journal|last1=Tirumalae|first1=Rajalakshmi|title=Psoriasiform dermatoses: Microscopic approach|journal=Indian Journal of Dermatology|volume=58|issue=4|year=2013|pages=290|issn=0019-5154|doi=10.4103/0019-5154.113945}}</ref>
* Mycosis fungoides pathological course may be divided into three main stages:<ref name="SongWillemze2013">{{cite journal|last1=Song|first1=Sophie X.|last2=Willemze|first2=Rein|last3=Swerdlow|first3=Steven H.|last4=Kinney|first4=Marsha C.|last5=Said|first5=Jonathan W.|title=Mycosis Fungoides|journal=American Journal of Clinical Pathology|volume=139|issue=4|year=2013|pages=466–490|issn=1943-7722|doi=10.1309/AJCPOBDP2OQAJ5BR}}</ref><ref name="VoraAnjaneyan2012">{{cite journal|last1=Vora|first1=Rita|last2=Anjaneyan|first2=Gopikrishnan|last3=Mubashir|first3=Syed|last4=Talavia|first4=Parag|title=Mycosis Fungoides: Tumour d′emblee|journal=Indian Dermatology Online Journal|volume=3|issue=2|year=2012|pages=122|issn=2229-5178|doi=10.4103/2229-5178.96709}}</ref><ref name="Tirumalae2013">{{cite journal|last1=Tirumalae|first1=Rajalakshmi|title=Psoriasiform dermatoses: Microscopic approach|journal=Indian Journal of Dermatology|volume=58|issue=4|year=2013|pages=290|issn=0019-5154|doi=10.4103/0019-5154.113945}}</ref>
:* Premycotic stage
:* Premycotic stage
:* Mycotic stage
:* [[Mycotic]] stage
:* Tumoros stage
:* Tumoros stage
* The premycotic stage  
* The premycotic stage  
:* Non-diagnostic and represented by chronic nonspecific dermatisis associated with psoriasiform changes in [[epidermis]]
:* Non-diagnostic and represented by chronic nonspecific dermatisis associated with psoriasiform changes in [[epidermis]]
*  The mycotic stage
*  The mycotic stage
:* Shows a [[polymorphous]] inflammatory infiltrate in the dermis that contains small numbers of frankly atypical [[lymphoid]] cells
:* Shows a [[polymorphous]] [[inflammatory]] infiltrate in the [[dermis]] that contains small numbers of frankly atypical [[lymphoid]] [[cells]]
:* These cells may line up individually along the epidermal basal layer
:* These cells may line up individually along the [[Epidermis (skin)|epidermal]] basal layer
:* The presence of spongiosis is highly suggestive of mycosis fungoides  
:* The presence of spongiosis is highly suggestive of mycosis fungoides  
* Tumoros stage
* Tumoros stage
:* Expansion of the [[dermis]] due to dense infiltration by medium sized [[lymphocytes]] that are typically characterized by a cerebroid nuclei.
:* Expansion of the [[dermis]] due to dense [[Infiltration (medical)|infiltration]] by medium sized [[lymphocytes]] that are typically characterized by a [[[cerebroid]] [[nuclei]].
 
<br style="clear:left" />
<gallery widths="200px">
Image:Sezary syndrome 0001.jpg| Sézary's disease
Image:Sézary's disease- PAS stain.jpg| 61-year-old man presented in 1972 with unrelenting pruritus of six months’ duration. On the right is his peripheral blood film stained with Periodic Acid-Schiff (PAS) showing a neoplastic T cell (Sézary cell).
Image:Hem1SezaryCell2.jpg| Pleomorphic abnormal T cell with the characteristic cerebriform nuclei (Peripheral blood - MGG stain)
Image:Cutaneous T-cell lymphoma - very high mag.jpg| Features: Nests of lymphocytes in the epidermis; "Pautrier microabscesses". Single lymphocytes in epidermis; "lymphocyte exocytosis". Short linear arrays of lymphocytes along the basal layer of the epidermis; "epidermotropism".
</gallery>
[[Image:Sezary syndrome 0001.jpg|200px|thumb|left]]


==Genetics==
==Genetics==
* Development of cutaneous T cell lymphoma is the result of multiple genetic mutations.<ref name="ShinMonti2007">{{cite journal|last1=Shin|first1=J.|last2=Monti|first2=S.|last3=Aires|first3=D. J.|last4=Duvic|first4=M.|last5=Golub|first5=T.|last6=Jones|first6=D. A.|last7=Kupper|first7=T. S.|title=Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome|journal=Blood|volume=110|issue=8|year=2007|pages=3015–3027|issn=0006-4971|doi=10.1182/blood-2006-12-061507}}</ref><ref name="WongMishra2011">{{cite journal|last1=Wong|first1=Henry K.|last2=Mishra|first2=Anjali|last3=Hake|first3=Timothy|last4=Porcu|first4=Pierluigi|title=Evolving Insights in the Pathogenesis and Therapy of Cutaneous T-cell lymphoma (Mycosis Fungoides and Sezary Syndrome)|journal=British Journal of Haematology|volume=155|issue=2|year=2011|pages=150–166|issn=00071048|doi=10.1111/j.1365-2141.2011.08852.x}}</ref><ref name="Wilcox2011">{{cite journal|last1=Wilcox|first1=Ryan A.|title=Cutaneous T-cell lymphoma: 2011 update on diagnosis, risk-stratification, and management|journal=American Journal of Hematology|volume=86|issue=11|year=2011|pages=928–948|issn=03618609|doi=10.1002/ajh.22139}}</ref><ref name="Whittaker2006">{{cite journal|last1=Whittaker|first1=Sean|title=Biological Insights into the Pathogenesis of Cutaneous T-Cell Lymphomas (CTCL)|journal=Seminars in Oncology|volume=33|year=2006|pages=3–6|issn=00937754|doi=10.1053/j.seminoncol.2005.12.015}}</ref><ref>{{Cite journal
* Development of mycosis fungoides [[disease]] is the result of multiple [[genetic mutations]].<ref name="ShinMonti2007">{{cite journal|last1=Shin|first1=J.|last2=Monti|first2=S.|last3=Aires|first3=D. J.|last4=Duvic|first4=M.|last5=Golub|first5=T.|last6=Jones|first6=D. A.|last7=Kupper|first7=T. S.|title=Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome|journal=Blood|volume=110|issue=8|year=2007|pages=3015–3027|issn=0006-4971|doi=10.1182/blood-2006-12-061507}}</ref><ref name="WongMishra2011">{{cite journal|last1=Wong|first1=Henry K.|last2=Mishra|first2=Anjali|last3=Hake|first3=Timothy|last4=Porcu|first4=Pierluigi|title=Evolving Insights in the Pathogenesis and Therapy of Cutaneous T-cell lymphoma (Mycosis Fungoides and Sezary Syndrome)|journal=British Journal of Haematology|volume=155|issue=2|year=2011|pages=150–166|issn=00071048|doi=10.1111/j.1365-2141.2011.08852.x}}</ref><ref name="Wilcox2011">{{cite journal|last1=Wilcox|first1=Ryan A.|title=Cutaneous T-cell lymphoma: 2011 update on diagnosis, risk-stratification, and management|journal=American Journal of Hematology|volume=86|issue=11|year=2011|pages=928–948|issn=03618609|doi=10.1002/ajh.22139}}</ref><ref name="Whittaker2006">{{cite journal|last1=Whittaker|first1=Sean|title=Biological Insights into the Pathogenesis of Cutaneous T-Cell Lymphomas (CTCL)|journal=Seminars in Oncology|volume=33|year=2006|pages=3–6|issn=00937754|doi=10.1053/j.seminoncol.2005.12.015}}</ref><ref>{{Cite journal
  | author = [[Henry K. Wong]]
  | author = [[Henry K. Wong]]
  | title = Novel biomarkers, dysregulated epigenetics, and therapy in cutaneous T-cell lymphoma
  | title = Novel biomarkers, dysregulated epigenetics, and therapy in cutaneous T-cell lymphoma
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}}</ref>
}}</ref>


OR
* Genes involved in the [[pathogenesis]] of mycosis fungoides include:<ref name="KarenkoHahtola2005">{{cite journal|last1=Karenko|first1=Leena|last2=Hahtola|first2=Sonja|last3=Päivinen|first3=Suvi|last4=Karhu|first4=Ritva|last5=Syrjä|first5=Sanna|last6=Kähkönen|first6=Marketta|last7=Nedoszytko|first7=Boguslaw|last8=Kytölä|first8=Soili|last9=Zhou|first9=Ying|last10=Blazevic|first10=Vesna|last11=Pesonen|first11=Maria|last12=Nevala|first12=Hanna|last13=Nupponen|first13=Nina|last14=Sihto|first14=Harri|last15=Krebs|first15=Inge|last16=Poustka|first16=Annemarie|last17=Roszkiewicz|first17=Jadwiga|last18=Saksela|first18=Kalle|last19=Peterson|first19=Pärt|last20=Visakorpi|first20=Tapio|last21=Ranki|first21=Annamari|title=Primary Cutaneous T-Cell Lymphomas Show a Deletion or Translocation AffectingNAV3, the HumanUNC-53Homologue|journal=Cancer Research|volume=65|issue=18|year=2005|pages=8101–8110|issn=0008-5472|doi=10.1158/0008-5472.CAN-04-0366}}</ref>
** [[Deletion (genetics)|Deletions]] or [[translocations]] involving a [[gene]], NAV3, at 12q2 (helicaselike activity)
** [[Deletion (genetics)|Deletion]] in 42 regions and [[amplification]] in 21 observed with meaningful amplifications of 8q (MYC) and 17q ([[STAT3]]) and [[Deletion (genetics)|deletions]] of 17p ([[TP53]]) and 10 ([[PTEN (gene)|PTEN]], FAS)<ref name="LinPasero2012">{{cite journal|last1=Lin|first1=Yea-Lih|last2=Pasero|first2=Philippe|title=Interference Between DNA Replication and Transcription as a Cause of Genomic Instability|journal=Current Genomics|volume=13|issue=1|year=2012|pages=65–73|issn=13892029|doi=10.2174/138920212799034767}}</ref>


* Genes involved in the pathogenesis of cutaneous T cell lymphoma include:
*Other [[Deletion (genetics)|deletion]] such as: [[ZEB1]], [[ARID1A]], DNMT3A, [[CDKN2A]], FAS, [[ATM]], [[CTCF]], [[STAT5B]], [[PRKCQ]] and [[somatic]] [[Mutation|mutations]] ([[NFKB2]], [[CD28]], [[RHOA]]) observed in [[gene]] [[sequencing]].<ref name="ChoiGoh2015">{{cite journal|last1=Choi|first1=Jaehyuk|last2=Goh|first2=Gerald|last3=Walradt|first3=Trent|last4=Hong|first4=Bok S|last5=Bunick|first5=Christopher G|last6=Chen|first6=Kan|last7=Bjornson|first7=Robert D|last8=Maman|first8=Yaakov|last9=Wang|first9=Tiffany|last10=Tordoff|first10=Jesse|last11=Carlson|first11=Kacie|last12=Overton|first12=John D|last13=Liu|first13=Kristina J|last14=Lewis|first14=Julia M|last15=Devine|first15=Lesley|last16=Barbarotta|first16=Lisa|last17=Foss|first17=Francine M|last18=Subtil|first18=Antonio|last19=Vonderheid|first19=Eric C|last20=Edelson|first20=Richard L|last21=Schatz|first21=David G|last22=Boggon|first22=Titus J|last23=Girardi|first23=Michael|last24=Lifton|first24=Richard P|title=Genomic landscape of cutaneous T cell lymphoma|journal=Nature Genetics|volume=47|issue=9|year=2015|pages=1011–1019|issn=1061-4036|doi=10.1038/ng.3356}}</ref>
** Deletions or translocations involving a gene, NAV3, at 12q2 (helicaselike activity)<ref name="KarenkoHahtola2005">{{cite journal|last1=Karenko|first1=Leena|last2=Hahtola|first2=Sonja|last3=Päivinen|first3=Suvi|last4=Karhu|first4=Ritva|last5=Syrjä|first5=Sanna|last6=Kähkönen|first6=Marketta|last7=Nedoszytko|first7=Boguslaw|last8=Kytölä|first8=Soili|last9=Zhou|first9=Ying|last10=Blazevic|first10=Vesna|last11=Pesonen|first11=Maria|last12=Nevala|first12=Hanna|last13=Nupponen|first13=Nina|last14=Sihto|first14=Harri|last15=Krebs|first15=Inge|last16=Poustka|first16=Annemarie|last17=Roszkiewicz|first17=Jadwiga|last18=Saksela|first18=Kalle|last19=Peterson|first19=Pärt|last20=Visakorpi|first20=Tapio|last21=Ranki|first21=Annamari|title=Primary Cutaneous T-Cell Lymphomas Show a Deletion or Translocation AffectingNAV3, the HumanUNC-53Homologue|journal=Cancer Research|volume=65|issue=18|year=2005|pages=8101–8110|issn=0008-5472|doi=10.1158/0008-5472.CAN-04-0366}}</ref>
** Deletion in 42 regions and amplification in 21 observed with meaningful amplifications of 8q (MYC) and 17q (STAT3) and deletions of 17p (TP53) and 10 (PTEN, FAS)<ref name="LinPasero2012">{{cite journal|last1=Lin|first1=Yea-Lih|last2=Pasero|first2=Philippe|title=Interference Between DNA Replication and Transcription as a Cause of Genomic Instability|journal=Current Genomics|volume=13|issue=1|year=2012|pages=65–73|issn=13892029|doi=10.2174/138920212799034767}}</ref>


** Other deletion such as ZEB1, ARID1A, DNMT3A, CDKN2A, FAS, ATM, CTCF, STAT5B, PRKCQ and somatic mutations (NFKB2, CD28, RHOA) observed in gene sequencing.<ref name="ChoiGoh2015">{{cite journal|last1=Choi|first1=Jaehyuk|last2=Goh|first2=Gerald|last3=Walradt|first3=Trent|last4=Hong|first4=Bok S|last5=Bunick|first5=Christopher G|last6=Chen|first6=Kan|last7=Bjornson|first7=Robert D|last8=Maman|first8=Yaakov|last9=Wang|first9=Tiffany|last10=Tordoff|first10=Jesse|last11=Carlson|first11=Kacie|last12=Overton|first12=John D|last13=Liu|first13=Kristina J|last14=Lewis|first14=Julia M|last15=Devine|first15=Lesley|last16=Barbarotta|first16=Lisa|last17=Foss|first17=Francine M|last18=Subtil|first18=Antonio|last19=Vonderheid|first19=Eric C|last20=Edelson|first20=Richard L|last21=Schatz|first21=David G|last22=Boggon|first22=Titus J|last23=Girardi|first23=Michael|last24=Lifton|first24=Richard P|title=Genomic landscape of cutaneous T cell lymphoma|journal=Nature Genetics|volume=47|issue=9|year=2015|pages=1011–1019|issn=1061-4036|doi=10.1038/ng.3356}}</ref>
* The development of cutaneous T cell lymphoma is the result of multiple genetic mutations such as (There is not a classic chromosomal translocation in cutaeous T cell lymphoma( MF and SS ) significant):<ref>{{Cite journal
 
* The development of cutaneous T cell lymphoma is the result of multiple genetic mutations such as:<ref>{{Cite journal
  | author = [[Leena Karenko]], [[Sonja Hahtola]], [[Suvi Paivinen]], [[Ritva Karhu]], [[Sanna Syrja]], [[Marketta Kahkonen]], [[Boguslaw Nedoszytko]], [[Soili Kytola]], [[Ying Zhou]], [[Vesna Blazevic]], [[Maria Pesonen]], [[Hanna Nevala]], [[Nina Nupponen]], [[Harri Sihto]], [[Inge Krebs]], [[Annemarie Poustka]], [[Jadwiga Roszkiewicz]], [[Kalle Saksela]], [[Part Peterson]], [[Tapio Visakorpi]] & [[Annamari Ranki]]
  | author = [[Leena Karenko]], [[Sonja Hahtola]], [[Suvi Paivinen]], [[Ritva Karhu]], [[Sanna Syrja]], [[Marketta Kahkonen]], [[Boguslaw Nedoszytko]], [[Soili Kytola]], [[Ying Zhou]], [[Vesna Blazevic]], [[Maria Pesonen]], [[Hanna Nevala]], [[Nina Nupponen]], [[Harri Sihto]], [[Inge Krebs]], [[Annemarie Poustka]], [[Jadwiga Roszkiewicz]], [[Kalle Saksela]], [[Part Peterson]], [[Tapio Visakorpi]] & [[Annamari Ranki]]
  | title = Primary cutaneous T-cell lymphomas show a deletion or translocation affecting NAV3, the human UNC-53 homologue
  | title = Primary cutaneous T-cell lymphomas show a deletion or translocation affecting NAV3, the human UNC-53 homologue
Line 102: Line 92:
  | pmid = 26192916
  | pmid = 26192916
}}</ref>
}}</ref>
 
*chromosomal instability has been noted. Losses on 1p, 10q, 13q, and 17p and gains of 4, 17q, and 18 have been identified <ref name="MaoOrchard2003">{{cite journal|last1=Mao|first1=Xin|last2=Orchard|first2=Guy|last3=Lillington|first3=Debra M.|last4=Russell-Jones|first4=Robin|last5=Young|first5=Bryan D.|last6=Whittaker|first6=Sean|title=Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma|journal=Genes, Chromosomes and Cancer|volume=37|issue=2|year=2003|pages=176–185|issn=10452257|doi=10.1002/gcc.10184}}</ref>
*There is not a classic chromosomal translocation in cutaeous T cell lymphoma( MF and SS ) significant
**[[Deletion (genetics)|Deletions]] and [[translocations]] in different [[chromosomes]] or [[chromosomal]] segments
chromosomal instability has been noted. Losses on 1p, 10q, 13q, and 17p and gains
**[[Chromosomal]] [[amplification]] of JunB at 19p12 observed in mycosis fungoides and Sezary syndrome.<ref name="MaoOrchard2003">{{cite journal|last1=Mao|first1=Xin|last2=Orchard|first2=Guy|last3=Lillington|first3=Debra M.|last4=Russell-Jones|first4=Robin|last5=Young|first5=Bryan D.|last6=Whittaker|first6=Sean|title=Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma|journal=Genes, Chromosomes and Cancer|volume=37|issue=2|year=2003|pages=176–185|issn=10452257|doi=10.1002/gcc.10184}}</ref>
of 4, 17q, and 18 have been identified <ref name="MaoOrchard2003">{{cite journal|last1=Mao|first1=Xin|last2=Orchard|first2=Guy|last3=Lillington|first3=Debra M.|last4=Russell-Jones|first4=Robin|last5=Young|first5=Bryan D.|last6=Whittaker|first6=Sean|title=Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma|journal=Genes, Chromosomes and Cancer|volume=37|issue=2|year=2003|pages=176–185|issn=10452257|doi=10.1002/gcc.10184}}</ref>
*deletions and translocations in different chromosomes or chromosomal segments
*Chromosomal amplification of JunB at 19p12 observed in mycosis fungoides and Sezary syndrome.<ref name="MaoOrchard2003">{{cite journal|last1=Mao|first1=Xin|last2=Orchard|first2=Guy|last3=Lillington|first3=Debra M.|last4=Russell-Jones|first4=Robin|last5=Young|first5=Bryan D.|last6=Whittaker|first6=Sean|title=Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma|journal=Genes, Chromosomes and Cancer|volume=37|issue=2|year=2003|pages=176–185|issn=10452257|doi=10.1002/gcc.10184}}</ref>


==References==
==References==

Latest revision as of 16:08, 30 May 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

Mycosis fungoides arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response. On microscopic histopathological analysis, atypical lymphoid cells, polymorphous inflammatory infiltrate in the dermis, and lymphocytes with cerebroid nuclei are characteristic findings of mycosis fungoides.

Pathophysiology

Microscopic Pathology

  • Mycosis fungoides pathological course may be divided into three main stages:[6][7][8]
  • Premycotic stage
  • Mycotic stage
  • Tumoros stage
  • The premycotic stage
  • Non-diagnostic and represented by chronic nonspecific dermatisis associated with psoriasiform changes in epidermis
  • The mycotic stage
  • Tumoros stage


Genetics

  • The development of cutaneous T cell lymphoma is the result of multiple genetic mutations such as (There is not a classic chromosomal translocation in cutaeous T cell lymphoma( MF and SS ) significant):[17][18][19][20]
  • chromosomal instability has been noted. Losses on 1p, 10q, 13q, and 17p and gains of 4, 17q, and 18 have been identified [21]

References

  1. ↑ Wilcox RA (January 2016). "Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management". Am. J. Hematol. 91 (1): 151–65. doi:10.1002/ajh.24233. PMC 4715621. PMID 26607183.
  2. ↑ Fuhlbrigge RC, Kieffer JD, Armerding D, Kupper TS (October 1997). "Cutaneous lymphocyte antigen is a specialized form of PSGL-1 expressed on skin-homing T cells". Nature. 389 (6654): 978–81. doi:10.1038/40166. PMID 9353122.
  3. ↑ 3.0 3.1 3.2 Foss, Francine M.; Girardi, Michael (2017). "Mycosis Fungoides and Sezary Syndrome". Hematology/Oncology Clinics of North America. 31 (2): 297–315. doi:10.1016/j.hoc.2016.11.008. ISSN 0889-8588.
  4. ↑ Bagherani, Nooshin; Smoller, Bruce R. (2016). "An overview of cutaneous T cell lymphomas". F1000Research. 5: 1882. doi:10.12688/f1000research.8829.1. ISSN 2046-1402.
  5. ↑ Mahalingam, Meera; Reddy, Vijaya B. (2015). "Mycosis Fungoides, Then and Now… Have We Travelled?". Advances In Anatomic Pathology. 22 (6): 376–383. doi:10.1097/PAP.0000000000000092. ISSN 1072-4109.
  6. ↑ Song, Sophie X.; Willemze, Rein; Swerdlow, Steven H.; Kinney, Marsha C.; Said, Jonathan W. (2013). "Mycosis Fungoides". American Journal of Clinical Pathology. 139 (4): 466–490. doi:10.1309/AJCPOBDP2OQAJ5BR. ISSN 1943-7722.
  7. ↑ Vora, Rita; Anjaneyan, Gopikrishnan; Mubashir, Syed; Talavia, Parag (2012). "Mycosis Fungoides: Tumour d′emblee". Indian Dermatology Online Journal. 3 (2): 122. doi:10.4103/2229-5178.96709. ISSN 2229-5178.
  8. ↑ Tirumalae, Rajalakshmi (2013). "Psoriasiform dermatoses: Microscopic approach". Indian Journal of Dermatology. 58 (4): 290. doi:10.4103/0019-5154.113945. ISSN 0019-5154.
  9. ↑ Shin, J.; Monti, S.; Aires, D. J.; Duvic, M.; Golub, T.; Jones, D. A.; Kupper, T. S. (2007). "Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome". Blood. 110 (8): 3015–3027. doi:10.1182/blood-2006-12-061507. ISSN 0006-4971.
  10. ↑ Wong, Henry K.; Mishra, Anjali; Hake, Timothy; Porcu, Pierluigi (2011). "Evolving Insights in the Pathogenesis and Therapy of Cutaneous T-cell lymphoma (Mycosis Fungoides and Sezary Syndrome)". British Journal of Haematology. 155 (2): 150–166. doi:10.1111/j.1365-2141.2011.08852.x. ISSN 0007-1048.
  11. ↑ Wilcox, Ryan A. (2011). "Cutaneous T-cell lymphoma: 2011 update on diagnosis, risk-stratification, and management". American Journal of Hematology. 86 (11): 928–948. doi:10.1002/ajh.22139. ISSN 0361-8609.
  12. ↑ Whittaker, Sean (2006). "Biological Insights into the Pathogenesis of Cutaneous T-Cell Lymphomas (CTCL)". Seminars in Oncology. 33: 3–6. doi:10.1053/j.seminoncol.2005.12.015. ISSN 0093-7754.
  13. ↑ Henry K. Wong (2013). "Novel biomarkers, dysregulated epigenetics, and therapy in cutaneous T-cell lymphoma". Discovery medicine. 16 (87): 71–78. PMID 23998443. Unknown parameter |month= ignored (help)
  14. ↑ Karenko, Leena; Hahtola, Sonja; Päivinen, Suvi; Karhu, Ritva; Syrjä, Sanna; Kähkönen, Marketta; Nedoszytko, Boguslaw; Kytölä, Soili; Zhou, Ying; Blazevic, Vesna; Pesonen, Maria; Nevala, Hanna; Nupponen, Nina; Sihto, Harri; Krebs, Inge; Poustka, Annemarie; Roszkiewicz, Jadwiga; Saksela, Kalle; Peterson, Pärt; Visakorpi, Tapio; Ranki, Annamari (2005). "Primary Cutaneous T-Cell Lymphomas Show a Deletion or Translocation AffectingNAV3, the HumanUNC-53Homologue". Cancer Research. 65 (18): 8101–8110. doi:10.1158/0008-5472.CAN-04-0366. ISSN 0008-5472.
  15. ↑ Lin, Yea-Lih; Pasero, Philippe (2012). "Interference Between DNA Replication and Transcription as a Cause of Genomic Instability". Current Genomics. 13 (1): 65–73. doi:10.2174/138920212799034767. ISSN 1389-2029.
  16. ↑ Choi, Jaehyuk; Goh, Gerald; Walradt, Trent; Hong, Bok S; Bunick, Christopher G; Chen, Kan; Bjornson, Robert D; Maman, Yaakov; Wang, Tiffany; Tordoff, Jesse; Carlson, Kacie; Overton, John D; Liu, Kristina J; Lewis, Julia M; Devine, Lesley; Barbarotta, Lisa; Foss, Francine M; Subtil, Antonio; Vonderheid, Eric C; Edelson, Richard L; Schatz, David G; Boggon, Titus J; Girardi, Michael; Lifton, Richard P (2015). "Genomic landscape of cutaneous T cell lymphoma". Nature Genetics. 47 (9): 1011–1019. doi:10.1038/ng.3356. ISSN 1061-4036.
  17. ↑ Leena Karenko, Sonja Hahtola, Suvi Paivinen, Ritva Karhu, Sanna Syrja, Marketta Kahkonen, Boguslaw Nedoszytko, Soili Kytola, Ying Zhou, Vesna Blazevic, Maria Pesonen, Hanna Nevala, Nina Nupponen, Harri Sihto, Inge Krebs, Annemarie Poustka, Jadwiga Roszkiewicz, Kalle Saksela, Part Peterson, Tapio Visakorpi & Annamari Ranki (2005). "Primary cutaneous T-cell lymphomas show a deletion or translocation affecting NAV3, the human UNC-53 homologue". Cancer research. 65 (18): 8101–8110. doi:10.1158/0008-5472.CAN-04-0366. PMID 16166283. Unknown parameter |month= ignored (help)
  18. ↑ Yaohua Zhang, Yang Wang, Richard Yu, Yuanshen Huang, Mingwan Su, Cheng Xiao, Magdalena Martinka, Jan P. Dutz, Xuejun Zhang, Zhizhong Zheng & Youwen Zhou (2012). "Molecular markers of early-stage mycosis fungoides". The Journal of investigative dermatology. 132 (6): 1698–1706. doi:10.1038/jid.2012.13. PMID 22377759. Unknown parameter |month= ignored (help)
  19. ↑ Alexander Ungewickell, Aparna Bhaduri, Eon Rios, Jason Reuter, Carolyn S. Lee, Angela Mah, Ashley Zehnder, Robert Ohgami, Shashikant Kulkarni, Randall Armstrong, Wen-Kai Weng, Dita Gratzinger, Mahkam Tavallaee, Alain Rook, Michael Snyder, Youn Kim & Paul A. Khavari (2015). "Genomic analysis of mycosis fungoides and Sezary syndrome identifies recurrent alterations in TNFR2". Nature genetics. 47 (9): 1056–1060. doi:10.1038/ng.3370. PMID 26258847. Unknown parameter |month= ignored (help)
  20. ↑ Jaehyuk Choi, Gerald Goh, Trent Walradt, Bok S. Hong, Christopher G. Bunick, Kan Chen, Robert D. Bjornson, Yaakov Maman, Tiffany Wang, Jesse Tordoff, Kacie Carlson, John D. Overton, Kristina J. Liu, Julia M. Lewis, Lesley Devine, Lisa Barbarotta, Francine M. Foss, Antonio Subtil, Eric C. Vonderheid, Richard L. Edelson, David G. Schatz, Titus J. Boggon, Michael Girardi & Richard P. Lifton (2015). "Genomic landscape of cutaneous T cell lymphoma". Nature genetics. 47 (9): 1011–1019. doi:10.1038/ng.3356. PMID 26192916. Unknown parameter |month= ignored (help)
  21. ↑ 21.0 21.1 Mao, Xin; Orchard, Guy; Lillington, Debra M.; Russell-Jones, Robin; Young, Bryan D.; Whittaker, Sean (2003). "Genetic alterations in primary cutaneous CD30+ anaplastic large cell lymphoma". Genes, Chromosomes and Cancer. 37 (2): 176–185. doi:10.1002/gcc.10184. ISSN 1045-2257.


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