Sézary's disease

DiseaseDisorder infobox
Name = Sézary disease
ICD10 = ICD10|C|84|1|c|81
ICD9 = ICD9|202.2
ICDO = ICDO|9701|3


Caption =
OMIM =
MedlinePlus =
eMedicineSubj = med
eMedicineTopic = 1541
eMedicine_mult = eMedicine2|derm|566 eMedicine2|med|3486
DiseasesDB = 8595
MeshID = D012751

Sézary's disease (often named Sézary syndrome) is a type of cutaneous lymphoma which was first described by Albert Sézary. [WhoNamedIt|synd|3594] The affected cells are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides.cite web | last = Cuneo A | first = Castoldi | title = Mycosis fungoidses/Sezary's syndrome | url=http://atlasgeneticsoncology.org/Anomalies/MycosFungID2039.html | accessdate = 2008-02-15 ] cite web | last = Thangavelu | first = Maya | title = Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome | url=http://bloodjournal.hematologylibrary.org/cgi/content/full/89/9/3371 | accessdate = 2008-02-15 ] There are currently no known causes of Sézary's disease.cite web | title = Causes and Symptoms | url=http://www.sezary.com/sezary-syndrome-causes.html | accessdate = 2008-02-15 ]

igns and symptoms

Sézary syndrome and mycosis fungoides are T-cell lymphomas whose primary manifestation is in the skin. The disease's origin is a peripheral CD4+ T-lymphocyte, although rarer CD8+/CD4- cases have been observed. Epidermotropism by neoplastic CD4+ lymphocytes with the formation of Pautrier's microabscesses is the hallmark sign of the disease. The dominant symptoms of the disease are:

# Generalized erythroderma
# Lymphadenopathy
# Atypical T-cells ("Sézary cells") in the peripheral blood
# Hepatosplenomegalycite web | last = Lorincz | first = A. I. | title = Sezary syndrome | url=http://www.gpnotebook.co.uk/simplepage.cfm?ID=584056846 | accessdate = 2008-02-15 ]

Diagnosis

Patients who have Sézary's disease often present with skin lesions that do not heal with normal medication.cite web | title = Diagnosis | url=http://www.sezary.com/sezary-syndrome-diagnosis.html | accessdate = 2008-02-15 ] 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. Finally, a biopsy of a skin lesion can be performed to rule out any other causes.

Treatment

Vorinostat (Zolinza) is a second-line drug for cutaneous T-cell lymphoma.Fact|date=February 2008 Treatments are often used in combination with phototherapy and chemotherapy.

Epidemiology

Mycosis fungoides is the most common form of cutaneous T-cell lymphoma. In the western population there are around 0.3 cases of Sezary syndrome per 100,000 people. Sézary disease is more common in males with a ratio of 2:1, and the mean age of diagnosis is between 55 and 60 years of age. Patients with Sézary disease have a median survival of 5 years.

References

External links

* [http://www.wadsworth.org/chemheme/heme/glass/slide_015_sezary.htm Illustration of Sezary cells]
* [http://www.academic.marist.edu/~jzmz/HematologyI/Lympholeukemia34.html Illustration of Sezary cells]
* [http://www.bium.univ-paris5.fr/sfhd/biographies/sezary.htm Biography of Sezary (in French)]
* [http://www.lymphomainfo.net/nhl/types/ctcl-mf.html Sezary Syndrome lymphoma information]
* [http://www.thedoctorsdoctor.com/diseases/sezary_syndrome.htm Doctor's doctor]
* [http://www.clfoundation.org Cutaneous Lymphoma Foundation ]
* [http://www.clinicaltrials.gov/ct/show/NCT00106431 Clinical Trial for Sezary Syndrome