| 词条 | Sézary disease |
| 释义 |
| name = Sézary disease | synonyms = Sézary's disease, Sézary('s) syndrome | image = Sezery2.jpg | caption = The bright red rash of Sezary syndrome | field = oncology, dermatology | pronounce = {{IPAc-en|ˌ|s|eɪ|ˌ|z|ɑː|ˈ|r|iː}} | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Sézary disease is a type of cutaneous lymphoma that was first described by Albert Sézary.[1] The affected cells are T-cells (so it is a T-cell lymphoma) that have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[2][3] There are currently no known causes of Sézary disease.[4] Signs and symptomsSézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin.[5] The disease's origin is a peripheral CD4+ T-lymphocyte,[2] although rarer CD8+/CD4- cases have been observed.[2] Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. Although the condition can affect people of all ages, it is commonly diagnosed in adults over age 60.[6][2] The dominant symptoms of the disease are:
DiagnosisThose who have Sézary disease often present with skin lesions that do not heal with normal medication.[10] A blood test generally reveals any change in the levels of lymphocytes in the blood, which is often associated with a cutaneous T-cell lymphoma.[10] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[10] The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences:[11]
TreatmentTreatment typically includes some combination of photodynamic therapy, radiation therapy, chemotherapy, and biologic therapy.[12] Treatments are often used in combination with phototherapy and chemotherapy, though pure chemotherapy is rarely used today.[2] No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.[13] Radiation therapyA number of types of radiation therapy may be used including total skin electron therapy.[23] While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin.[23] It is only available at a few institutions.[14] ChemotherapyRomidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma.[15] Mogamulizumab has been approved in Japan and waiting FDA approval in the United States. There are dozens of clinical trials, with a few in Phase III. EpidemiologyIn the Western population, there are around 3 cases of Sézary syndrome per 1,000,000 people.[2] Sézary disease is more common in males with a ratio of 2:1,[2] and the mean age of diagnosis is between 55 and 60 years of age.[2][7] See also
References1. ^{{WhoNamedIt|synd|3594|Sézary's cell}} 2. ^1 2 3 4 5 6 7 8 9 10 {{cite journal | last = Cuneo A | first = Castoldi | title = Mycosis fungoidses/Sezary's syndrome | url=http://atlasgeneticsoncology.org/Anomalies/MycosFungID2039.html | accessdate = 2008-02-15 |journal=Atlas Genet Cytogenet Oncol Haematol. 2005;9(3):242-243. |year=2005}} 3. ^{{cite journal |vauthors=Thangavelu M, Finn WG, Yelavarthi KK, Roenigk Jr HH, Samuelson E, Peterson L, Kuzel TM, Rosen ST |title=Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome |journal=Blood |volume=89 |issue=9 |pages=3371–7 |year=May 1997 |url=http://www.bloodjournal.org/content/89/9/3371.full |pmid=9129044}} 4. ^{{cite web | title = Causes and Symptoms | url=http://www.sezary.com/sezary-syndrome-causes.html | accessdate = 2008-02-15 }} 5. ^{{cite book | last = Cerroni | first = Lorenzo | authorlink = |author2=Kevin Gatter |author3=Helmut Kerl | title = An illustrated guide to Skin Lymphomas | publisher = Blackwell Publishing | year = 2005 | location = Malden, Massachusetts | pages = 39 | url = | doi = | id = | isbn = 978-1-4051-1376-2 }} 6. ^{{cite web|title=Sezary syndrome|url=https://rarediseases.info.nih.gov/diseases/7629/sezary-syndrome|website=genetic and rare diseases information center|accessdate=17 April 2018}} 7. ^1 {{cite web | last = Lorincz | first = A. I. | title = Sezary syndrome | url=http://www.gpnotebook.co.uk/simplepage.cfm?ID=584056846 | accessdate = 2008-02-15 }} 8. ^{{Cite journal|last=Fragkos|first=Konstantinos C.|title=Plantar keratoderma of Sézary syndrome|journal=Clinical Case Reports|volume=5|issue=10|language=en|pages=1726–1727|doi=10.1002/ccr3.1168|pmid=29026585|pmc=5628207|issn=2050-0904|year=2017}} 9. ^{{Cite journal|last=Martin|first=Stephanie J.|last2=Duvic|first2=Madeleine|date=2012-10-01|title=Prevalence and treatment of palmoplantar keratoderma and tinea pedis in patients with Sézary syndrome|journal=International Journal of Dermatology|language=en|volume=51|issue=10|pages=1195–1198|doi=10.1111/j.1365-4632.2011.05204.x|pmid=22994666|issn=1365-4632}} 10. ^1 2 {{cite web | title = Diagnosis | url=http://www.sezary.com/sezary-syndrome-diagnosis.html |access-date= 2008-02-15 }} 11. ^{{Cite book|title=Clinician's Guide to Mycosis Fungoides|journal=Bulletin of the New York Academy of Medicine|volume=35|issue=2|last=Beigi|first=Pooya Khan Mohammad|date=2017|publisher=Springer International Publishing|isbn=9783319479064|pages=13–18|language=en|doi=10.1007/978-3-319-47907-1_4|pmid=13618707|pmc = 1806090|chapter = Diagnosis and Management}} 12. ^{{cite web|title=Mycosis Fungoides (Including Sézary Syndrome) Treatment|url=https://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq#link/_50|website=National Cancer Institute|accessdate=1 December 2017|language=en|date=7 September 2017}} 13. ^{{cite book | last = Cerroni | first = Lorenzo | authorlink = |author2=Kevin Gatter |author3=Helmut Kerl | title = An illustrated guide to Skin Lymphomas | publisher = Blackwell Publishing | year = 2005 | location = Malden, Massachusetts | pages = 41 | url = | doi = | id = | isbn = 978-1-4051-1376-2 }} 14. ^1 2 {{cite web|title=Mycosis Fungoides (Including Sézary Syndrome) Treatment|url=https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq#link/_449_toc|website=National Cancer Institute|accessdate=1 December 2017|language=en|date=12 July 2017}} 15. ^{{cite journal|last1=Jawed|first1=SI|last2=Myskowski|first2=PL|last3=Horwitz|first3=S|last4=Moskowitz|first4=A|last5=Querfeld|first5=C|title=Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.|journal=Journal of the American Academy of Dermatology|date=February 2014|volume=70|issue=2|pages=223.e1–17; quiz 240–2 |doi=10.1016/j.jaad.2013.08.033|pmid=24438970}} External links{{Medical resources| ICD10 = {{ICD10|C|84|1|c|81}} | ICD9 = {{ICD9|202.2}} | ICDO = {{ICDO|9701|3}} | OMIM = | MedlinePlus = | eMedicineSubj = med | eMedicineTopic = 1541 | eMedicine_mult = {{eMedicine2|derm|566}} {{eMedicine2|med|3486}} | DiseasesDB = 8595 | MeshID = D012751 }}
3 : Lymphoma|Lymphoid-related cutaneous conditions|Syndromes |
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