Sézary disease

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Sézary disease
Synonyms Sézary's disease, Sézary('s) syndrome
Sezery2.jpg
The bright red rash of Sezary syndrome
Pronunciation
Specialty oncology, dermatology

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 symptoms[edit]

Buttock cell: pleomorphic abnormal T cell with the characteristic cerebriform nuclei (Peripheral blood - MGG stain).

Sézary 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.[2] The dominant symptoms of the disease are:

  1. Generalized erythroderma[2]
  2. Lymphadenopathy[2]
  3. Atypical T-cells ("Sézary cells") in the peripheral blood[2]
  4. Hepatosplenomegaly[6]
  5. Palmoplantar keratoderma[7][8]

Diagnosis[edit]

Sézary syndrome in a 61-year-old man presenting in 1972 with unrelenting itchiness 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).

Those who have Sézary disease often present with skin lesions that do not heal with normal medication.[9] 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.[9] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[9]

The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences[10]:

  1. More monotonous cellular infiltrates (large, clustered atypical pagetoid cells) in Sézary syndrome
  2. Sometimes absent epidermotropism
  3. Increased lymph node involvement with infiltrates of Sézary syndrome.

Treatment[edit]

Treatment typically includes some combination of photodynamic therapy, radiation therapy, chemotherapy, and biologic therapy.[11]

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.[12]

Radiation therapy[edit]

A number of types of radiation therapy may be used including total skin electron therapy.[13] While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin.[13] It is only avaliable at a few institutions.[13]

Chemotherapy[edit]

Romidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma.[14] 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.

Epidemiology[edit]

In the Western population, there are around 0.3 cases of Sézary syndrome per 100,000 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][6]

See also[edit]

References[edit]

  1. ^ Sézary's cell at Who Named It?
  2. ^ a b c d e f g h i j k Cuneo A, Castoldi (2005). "Mycosis fungoidses/Sezary's syndrome". Atlas Genet Cytogenet Oncol Haematol. 2005;9(3):242-243. Retrieved 2008-02-15. 
  3. ^ Thangavelu M, Finn WG, Yelavarthi KK, Roenigk Jr HH, Samuelson E, Peterson L, Kuzel TM, Rosen ST (May 1997). "Recurring Structural Chromosome Abnormalities in Peripheral Blood Lymphocytes of Patients With Mycosis Fungoides/Sézary Syndrome". Blood. 89 (9): 3371–7. PMID 9129044. 
  4. ^ "Causes and Symptoms". Retrieved 2008-02-15. 
  5. ^ Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39. ISBN 978-1-4051-1376-2. 
  6. ^ a b Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15. 
  7. ^ Fragkos, Konstantinos C. "Plantar keratoderma of Sézary syndrome". Clinical Case Reports: n/a–n/a. doi:10.1002/ccr3.1168. ISSN 2050-0904. 
  8. ^ Martin, Stephanie J.; Duvic, Madeleine (2012-10-01). "Prevalence and treatment of palmoplantar keratoderma and tinea pedis in patients with Sézary syndrome". International Journal of Dermatology. 51 (10): 1195–1198. doi:10.1111/j.1365-4632.2011.05204.x. ISSN 1365-4632. 
  9. ^ a b c "Diagnosis". Retrieved 2008-02-15. 
  10. ^ Beigi, Pooya Khan Mohammad (2017). Clinician's Guide to Mycosis Fungoides. Springer International Publishing. pp. 13–18. doi:10.1007/978-3-319-47907-1_4. ISBN 9783319479064. 
  11. ^ "Mycosis Fungoides (Including Sézary Syndrome) Treatment". National Cancer Institute. 7 September 2017. Retrieved 1 December 2017. 
  12. ^ Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 41. ISBN 978-1-4051-1376-2. 
  13. ^ a b c "Mycosis Fungoides (Including Sézary Syndrome) Treatment". National Cancer Institute. 12 July 2017. Retrieved 1 December 2017. 
  14. ^ Jawed, SI; Myskowski, PL; Horwitz, S; Moskowitz, A; Querfeld, C (February 2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions". Journal of the American Academy of Dermatology. 70 (2): 223.e1–17; quiz 240–2. doi:10.1016/j.jaad.2013.08.033. PMID 24438970. 

External links[edit]

Classification
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External resources