Sezary syndrome pathophysiology: Difference between revisions
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===Pathogenesis=== | ===Pathogenesis=== | ||
*The exact pathogenesis of | * The exact [[pathogenesis]] of Sezary syndrome is not fully understood.<ref name="SauliteHoetzenecker2016">{{cite journal|last1=Saulite|first1=Ieva|last2=Hoetzenecker|first2=Wolfram|last3=Weidinger|first3=Stephan|last4=Cozzio|first4=Antonio|last5=Guenova|first5=Emmanuella|last6=Wehkamp|first6=Ulrike|title=Sézary Syndrome and Atopic Dermatitis: Comparison of Immunological Aspects and Targets|journal=BioMed Research International|volume=2016|year=2016|pages=1–15|issn=2314-6133|doi=10.1155/2016/9717530}}</ref> | ||
* Sezary syndrome is an [[Erythroderma|erythrodermic]] [[cutaneous]] [[T-cell lymphoma]] with a [[leukemic]] involvement of [[malignant]] [[T cell|T cells]].<ref name="pmid12859741">{{cite journal |vauthors=Balfour EM, Glusac EJ, Heald P, Talley LL, Smoller BR |title=Sezary syndrome: cutaneous immunoperoxidase double-labeling technique demonstrates CD4/CD8 ratio non-specificity |journal=J. Cutan. Pathol. |volume=30 |issue=7 |pages=437–42 |date=August 2003 |pmid=12859741 |doi= |url=}}</ref> | |||
* | * [[Cutaneous]] [[T-cell lymphoma|T cell lymphoma]] arises from [[T-cell]] [[Lymphocyte|lymphocytes]], which are normally involved in the [[Cell (biology)|cell]] mediated [[immune]] response.<ref name="pmid26607183">{{cite journal |vauthors=Wilcox RA |title=Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=1 |pages=151–65 |date=January 2016 |pmid=26607183 |pmc=4715621 |doi=10.1002/ajh.24233 |url=}}</ref> | ||
*[ | * Sezary syndrome's [[Patient|patients]] have [[Immunodeficiency|suppressed]] [[Immunity (medical)|immunity]], as the [[malignant]] [[Cell (biology)|cells]] produce type-2, [[T cell|T-cell]] ([[T helper cell|Th2]]) [[Cytokine|cytokines]] which suppress [[T helper cell|Th1]] [[Immunity (medical)|immunity]] by decreasing the production of [[Interleukin 12|IL-12]]. The role of [[Interleukin 12|IL-12]] is to stimulate the production of [[interferon]] [[gamma]] and [[tumor necrosis factor-alpha]] ([[TNF-alpha|TNF-a]]), thus protecting against [[Tumor|tumors]].<ref name="SauliteHoetzenecker2016">{{cite journal|last1=Saulite|first1=Ieva|last2=Hoetzenecker|first2=Wolfram|last3=Weidinger|first3=Stephan|last4=Cozzio|first4=Antonio|last5=Guenova|first5=Emmanuella|last6=Wehkamp|first6=Ulrike|title=Sézary Syndrome and Atopic Dermatitis: Comparison of Immunological Aspects and Targets|journal=BioMed Research International|volume=2016|year=2016|pages=1–15|issn=2314-6133|doi=10.1155/2016/9717530}}</ref> | ||
* The [[tumor]] [[Cell (biology)|cells]] originate from [[memory T cells]] or [[skin]] homing [[CD4+ T cells]] expressing [[cutaneous]] [[lymphocyte]] [[antigen]] (CLA) and [[chemokine]] [[receptors]] [[CCR4]] and CCR7.<ref name="pmid9353122">{{cite journal |vauthors=Fuhlbrigge RC, Kieffer JD, Armerding D, Kupper TS |title=Cutaneous lymphocyte antigen is a specialized form of PSGL-1 expressed on skin-homing T cells |journal=Nature |volume=389 |issue=6654 |pages=978–81 |date=October 1997 |pmid=9353122 |doi=10.1038/40166 |url=}}</ref> | |||
*[ | * A few [[Patient|patients]] of Sezary syndrome are associated with human [[T cell|T]]-lymphotropic virus types 1 and 2 (HTLV1 and HTLV2) In Japan, Caribbean islands, and the Middle East.<ref name="pmid24982574">{{cite journal |vauthors=Graham RL, Burch M, Krause JR |title=Adult T-cell leukemia/lymphoma |journal=Proc (Bayl Univ Med Cent) |volume=27 |issue=3 |pages=235–8 |date=July 2014 |pmid=24982574 |pmc=4059578 |doi= |url=}}</ref><ref name="pmid21145619">{{cite journal |vauthors=Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, Wood G, Demierre MF, Pittelkow M, Wilson LD, Pinter-Brown L, Advani R, Parker S, Kim EJ, Junkins-Hopkins JM, Foss F, Cacchio P, Duvic M |title=Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC) |journal=J. Am. Acad. Dermatol. |volume=64 |issue=2 |pages=352–404 |date=February 2011 |pmid=21145619 |doi=10.1016/j.jaad.2010.08.037 |url=}}</ref> | ||
* | * In Sezary syndrome's [[Patient|patients]] [[blood]] [[flow]] cytometry shown [[measurement]] of the [[CD4+]]CD26- population of [[Lymphocyte|lymphocytes]] is a valuable tool for monitoring and CD26 seen in >90% of [[Patient|patients]] and [[CD7]] loss in 50% of [[patients]]. <ref name="pmid16112348">{{cite journal |vauthors=Introcaso CE, Hess SD, Kamoun M, Ubriani R, Gelfand JM, Rook AH |title=Association of change in clinical status and change in the percentage of the CD4+CD26- lymphocyte population in patients with Sézary syndrome |journal=J. Am. Acad. Dermatol. |volume=53 |issue=3 |pages=428–34 |date=September 2005 |pmid=16112348 |doi=10.1016/j.jaad.2005.06.001 |url=}}</ref><ref name="GorczycaWeisberger2002">{{cite journal|last1=Gorczyca|first1=Wojciech|last2=Weisberger|first2=James|last3=Liu|first3=Z.|last4=Tsang|first4=Patricia|last5=Hossein|first5=Monowar|last6=Wu|first6=C. Daniel|last7=Dong|first7=Henry|last8=Wong|first8=John Y.L.|last9=Tugulea|first9=Sorina|last10=Dee|first10=Simpson|last11=Melamed|first11=Myron R.|last12=Darzynkiewicz|first12=Zbigniew|title=An approach to diagnosis of T-cell lymphoproliferative disorders by flow cytometry|journal=Cytometry|volume=50|issue=3|year=2002|pages=177–190|issn=0196-4763|doi=10.1002/cyto.10003}}</ref> | ||
* | * In majority of Sezary syndrome's patients peripheral [[blood]] [[T cell]] seen [[CD3 (immunology)|CD3+]], [[CD4|CD4+]], [[CD7|CD7-]], [[CD26]]-, [[CD8|CD8-]].<ref name="pmid28802499">{{cite journal |vauthors=Pulitzer M |title=Cutaneous T-cell Lymphoma |journal=Clin. Lab. Med. |volume=37 |issue=3 |pages=527–546 |date=September 2017 |pmid=28802499 |pmc=5710803 |doi=10.1016/j.cll.2017.06.006 |url=}}</ref> | ||
* In some Sezary syndrome [[Patient|patients]] [[CD8|CD8+]] [[phenotype]] seen with [[retroviral]] [[Infection|infections]] ([[Human T-lymphotropic virus|HTLV-1]]/2 or HIV).<ref name="pmid15841167">{{cite journal |vauthors=Kim EJ, Hess S, Richardson SK, Newton S, Showe LC, Benoit BM, Ubriani R, Vittorio CC, Junkins-Hopkins JM, Wysocka M, Rook AH |title=Immunopathogenesis and therapy of cutaneous T cell lymphoma |journal=J. Clin. Invest. |volume=115 |issue=4 |pages=798–812 |date=April 2005 |pmid=15841167 |pmc=1070436 |doi=10.1172/JCI24826 |url=}}</ref><ref name="KashanchiCoelho-dos-Reis2013">{{cite journal|last1=Kashanchi|first1=Fatah|last2=Coelho-dos-Reis|first2=Jordana Grazziela Alves|last3=Passos|first3=Livia|last4=Duarte|first4=Mariana Costa|last5=Araújo|first5=Marcelo Grossi|last6=Campi-Azevedo|first6=Ana Carolina|last7=Teixeira-Carvalho|first7=Andréa|last8=Peruhype-Magalhães|first8=Vanessa|last9=Trindade|first9=Bruno Caetano|last10=dos Santos Dias|first10=Raquel|last11=Martins|first11=Marina Lobato|last12=Carneiro-Proietti|first12=Anna Barbara de Freitas|last13=Guedes|first13=Antônio Carlos|last14=Gonçalves|first14=Denise Utsch|last15=Martins-Filho|first15=Olindo Assis|title=Immunological Profile of HTLV-1-Infected Patients Associated with Infectious or Autoimmune Dermatological Disorders|journal=PLoS Neglected Tropical Diseases|volume=7|issue=7|year=2013|pages=e2328|issn=1935-2735|doi=10.1371/journal.pntd.0002328}}</ref><ref name="Myskowski1991">{{cite journal|last1=Myskowski|first1=Patricia L.|title=Cutaneous T-Cell Lymphoma and Human Immunodeficiency Virus|journal=Archives of Dermatology|volume=127|issue=7|year=1991|pages=1045|issn=0003-987X|doi=10.1001/archderm.1991.01680060119017}}</ref> | |||
* Sezary syndrome cells are [[T-cells]] that have pathological quantities of [[Mucopolysaccharide|mucopolysaccharides]]. | |||
* When Sezary cells move from the [[blood]] into the [[skin]], the skin problem is seen and Sezary cells are finded in [[rash]]. | |||
* In sezary syndrome maybe one or more [[chromosomal]] [[abnormalities]], such as the loss or gain of [[Genetics|genetic]].<ref name="pmid271214732">{{cite journal |vauthors=Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ |title=Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome |journal=Blood |volume=127 |issue=26 |pages=3387–97 |date=June 2016 |pmid=27121473 |doi=10.1182/blood-2016-02-699843 |url=}}</ref><ref name="pmid266071832">{{cite journal |vauthors=Wilcox RA |title=Cutaneous T-cell lymphoma: 2016 update on diagnosis, risk-stratification, and management |journal=Am. J. Hematol. |volume=91 |issue=1 |pages=151–65 |date=January 2016 |pmid=26607183 |pmc=4715621 |doi=10.1002/ajh.24233 |url=}}</ref> | |||
[[Image:Sézary's disease- PAS stain.jpg|200px|thumb|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).]] | |||
==Genetics== | ==Genetics== | ||
Revision as of 16:30, 13 February 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: {{S.G.}]
Overview
The exact pathogenesis of [disease name] is not fully understood.
OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.
Pathophysiology
Physiology
The normal physiology of [name of process] can be understood as follows:
Pathogenesis
- The exact pathogenesis of Sezary syndrome is not fully understood.[1]
- Sezary syndrome is an erythrodermic cutaneous T-cell lymphoma with a leukemic involvement of malignant T cells.[2]
- Cutaneous T cell lymphoma arises from T-cell lymphocytes, which are normally involved in the cell mediated immune response.[3]
- Sezary syndrome's patients have suppressed immunity, as the malignant cells produce type-2, T-cell (Th2) cytokines which suppress Th1 immunity by decreasing the production of IL-12. The role of IL-12 is to stimulate the production of interferon gamma and tumor necrosis factor-alpha (TNF-a), thus protecting against tumors.[1]
- The tumor cells originate from memory T cells or skin homing CD4+ T cells expressing cutaneous lymphocyte antigen (CLA) and chemokine receptors CCR4 and CCR7.[4]
- A few patients of Sezary syndrome are associated with human T-lymphotropic virus types 1 and 2 (HTLV1 and HTLV2) In Japan, Caribbean islands, and the Middle East.[5][6]
- In Sezary syndrome's patients blood flow cytometry shown measurement of the CD4+CD26- population of lymphocytes is a valuable tool for monitoring and CD26 seen in >90% of patients and CD7 loss in 50% of patients. [7][8]
- In majority of Sezary syndrome's patients peripheral blood T cell seen CD3+, CD4+, CD7-, CD26-, CD8-.[9]
- In some Sezary syndrome patients CD8+ phenotype seen with retroviral infections (HTLV-1/2 or HIV).[10][11][12]
- Sezary syndrome cells are T-cells that have pathological quantities of mucopolysaccharides.
- When Sezary cells move from the blood into the skin, the skin problem is seen and Sezary cells are finded in rash.
- In sezary syndrome maybe one or more chromosomal abnormalities, such as the loss or gain of genetic.[13][14]
Genetics
[Disease name] is transmitted in [mode of genetic transmission] pattern.
OR
Genes involved in the pathogenesis of [disease name] include:
- [Gene1]
- [Gene2]
- [Gene3]
OR
The development of [disease name] is the result of multiple genetic mutations such as:
- [Mutation 1]
- [Mutation 2]
- [Mutation 3]
Associated Conditions
Conditions associated with [disease name] include:
- [Condition 1]
- [Condition 2]
- [Condition 3]
Gross Pathology
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
Microscopic Pathology
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
References
- ↑ 1.0 1.1 Saulite, Ieva; Hoetzenecker, Wolfram; Weidinger, Stephan; Cozzio, Antonio; Guenova, Emmanuella; Wehkamp, Ulrike (2016). "Sézary Syndrome and Atopic Dermatitis: Comparison of Immunological Aspects and Targets". BioMed Research International. 2016: 1–15. doi:10.1155/2016/9717530. ISSN 2314-6133.
- ↑ Balfour EM, Glusac EJ, Heald P, Talley LL, Smoller BR (August 2003). "Sezary syndrome: cutaneous immunoperoxidase double-labeling technique demonstrates CD4/CD8 ratio non-specificity". J. Cutan. Pathol. 30 (7): 437–42. PMID 12859741.
- ↑ 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.
- ↑ 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.
- ↑ Graham RL, Burch M, Krause JR (July 2014). "Adult T-cell leukemia/lymphoma". Proc (Bayl Univ Med Cent). 27 (3): 235–8. PMC 4059578. PMID 24982574.
- ↑ Olsen EA, Rook AH, Zic J, Kim Y, Porcu P, Querfeld C, Wood G, Demierre MF, Pittelkow M, Wilson LD, Pinter-Brown L, Advani R, Parker S, Kim EJ, Junkins-Hopkins JM, Foss F, Cacchio P, Duvic M (February 2011). "Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)". J. Am. Acad. Dermatol. 64 (2): 352–404. doi:10.1016/j.jaad.2010.08.037. PMID 21145619.
- ↑ Introcaso CE, Hess SD, Kamoun M, Ubriani R, Gelfand JM, Rook AH (September 2005). "Association of change in clinical status and change in the percentage of the CD4+CD26- lymphocyte population in patients with Sézary syndrome". J. Am. Acad. Dermatol. 53 (3): 428–34. doi:10.1016/j.jaad.2005.06.001. PMID 16112348.
- ↑ Gorczyca, Wojciech; Weisberger, James; Liu, Z.; Tsang, Patricia; Hossein, Monowar; Wu, C. Daniel; Dong, Henry; Wong, John Y.L.; Tugulea, Sorina; Dee, Simpson; Melamed, Myron R.; Darzynkiewicz, Zbigniew (2002). "An approach to diagnosis of T-cell lymphoproliferative disorders by flow cytometry". Cytometry. 50 (3): 177–190. doi:10.1002/cyto.10003. ISSN 0196-4763.
- ↑ Pulitzer M (September 2017). "Cutaneous T-cell Lymphoma". Clin. Lab. Med. 37 (3): 527–546. doi:10.1016/j.cll.2017.06.006. PMC 5710803. PMID 28802499.
- ↑ Kim EJ, Hess S, Richardson SK, Newton S, Showe LC, Benoit BM, Ubriani R, Vittorio CC, Junkins-Hopkins JM, Wysocka M, Rook AH (April 2005). "Immunopathogenesis and therapy of cutaneous T cell lymphoma". J. Clin. Invest. 115 (4): 798–812. doi:10.1172/JCI24826. PMC 1070436. PMID 15841167.
- ↑ Kashanchi, Fatah; Coelho-dos-Reis, Jordana Grazziela Alves; Passos, Livia; Duarte, Mariana Costa; Araújo, Marcelo Grossi; Campi-Azevedo, Ana Carolina; Teixeira-Carvalho, Andréa; Peruhype-Magalhães, Vanessa; Trindade, Bruno Caetano; dos Santos Dias, Raquel; Martins, Marina Lobato; Carneiro-Proietti, Anna Barbara de Freitas; Guedes, Antônio Carlos; Gonçalves, Denise Utsch; Martins-Filho, Olindo Assis (2013). "Immunological Profile of HTLV-1-Infected Patients Associated with Infectious or Autoimmune Dermatological Disorders". PLoS Neglected Tropical Diseases. 7 (7): e2328. doi:10.1371/journal.pntd.0002328. ISSN 1935-2735.
- ↑ Myskowski, Patricia L. (1991). "Cutaneous T-Cell Lymphoma and Human Immunodeficiency Virus". Archives of Dermatology. 127 (7): 1045. doi:10.1001/archderm.1991.01680060119017. ISSN 0003-987X.
- ↑ Woollard WJ, Pullabhatla V, Lorenc A, Patel VM, Butler RM, Bayega A, Begum N, Bakr F, Dedhia K, Fisher J, Aguilar-Duran S, Flanagan C, Ghasemi AA, Hoffmann RM, Castillo-Mosquera N, Nuttall EA, Paul A, Roberts CA, Solomonidis EG, Tarrant R, Yoxall A, Beyers CZ, Ferreira S, Tosi I, Simpson MA, de Rinaldis E, Mitchell TJ, Whittaker SJ (June 2016). "Candidate driver genes involved in genome maintenance and DNA repair in Sézary syndrome". Blood. 127 (26): 3387–97. doi:10.1182/blood-2016-02-699843. PMID 27121473.
- ↑ 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.