Sezary syndrome pathophysiology: Difference between revisions

Jump to navigation Jump to search
Line 53: Line 53:
==Genetics==
==Genetics==
[Disease name] is transmitted in [mode of genetic transmission] pattern.
[Disease name] is transmitted in [mode of genetic transmission] pattern.
 
*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>
*[[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>
**[[Deletion (genetics)|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>


OR
OR

Revision as of 18:51, 13 February 2019

Sezary syndrome Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Sezary syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Sezary syndrome pathophysiology On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Sezary syndrome pathophysiology

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Sezary syndrome pathophysiology

CDC on Sezary syndrome pathophysiology

Sezary syndrome pathophysiology in the news

Blogs on Sezary syndrome pathophysiology

Directions to Hospitals Treating Psoriasis

Risk calculators and risk factors for Sezary syndrome pathophysiology

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

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

[Disease name] is transmitted in [mode of genetic transmission] pattern.

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

References

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.
  12. 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.
  13. 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.
  14. 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.
  15. 15.0 15.1 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.
  16. Arafah M, Zaidi SN, Kfoury HK, Al Rikabi A, Al Ghamdi K (March 2012). "The Histological Spectrum of Early Mycosis Fungoides: A Study of 58 Saudi Arab patients". Oman Med J. 27 (2): 134–9. doi:10.5001/omj.2012.28. PMC 3321345. PMID 22496939.
  17. Chu AC, Morris JF (May 1989). "Sézary cell morphology induced in peripheral blood lymphocytes: re-evaluation". Blood. 73 (6): 1603–7. PMID 2653459.
  18. Vonderheid EC, Bernengo MG, Burg G, Duvic M, Heald P, Laroche L, Olsen E, Pittelkow M, Russell-Jones R, Takigawa M, Willemze R (January 2002). "Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas". J. Am. Acad. Dermatol. 46 (1): 95–106. PMID 11756953.
  19. Guitart J (September 2005). "Beyond clonal detection: defining the T-cell clone". Arch Dermatol. 141 (9): 1159–60. doi:10.1001/archderm.141.9.1159. PMID 16172316.
  20. Kirsch IR, Watanabe R, O'Malley JT, Williamson DW, Scott LL, Elco CP, Teague JE, Gehad A, Lowry EL, LeBoeuf NR, Krueger JG, Robins HS, Kupper TS, Clark RA (October 2015). "TCR sequencing facilitates diagnosis and identifies mature T cells as the cell of origin in CTCL". Sci Transl Med. 7 (308): 308ra158. doi:10.1126/scitranslmed.aaa9122. PMC 4765389. PMID 26446955.

Template:WH Template:WS