Sézary disease

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Sézary syndrome
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Sézary disease
Other namesSézary's disease, Sézary('s) syndrome
The bright red rash of Sezary syndrome
Pronunciation
SpecialtyOncology, dermatology

Sézary disease, or Sézary syndrome,

mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.[3][4]

Signs and symptoms

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

Sézary disease and

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.[7][3]
The dominant signs and symptoms of the disease are:

  1. Generalized erythroderma – redness of the skin[3]
  2. Lymphadenopathy – swollen, enlarged lymph nodes[3]
  3. Atypical T cells – malignant lymphocytes known as "Sézary cells" seen in the peripheral blood with typical cerebriform nuclei (brain-shaped, convoluted nuclei)[8][3]
  4. Hepatosplenomegaly– enlarged liver and spleen[9]
  5. Palmoplantar keratoderma – thickening of the palms of the hands, and soles of the feet[10][11]

Diagnosis

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 skin lesions that do not heal with normal medication.[12] 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.[12] Finally, a biopsy of a skin lesion can be performed to rule out any other causes.[12]

The immunohistochemical features are very similar to those presented in mycosis fungoides except for the following differences:[13]

  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

Treatment typically includes some combination of

biologic therapy.[14]

Treatments are often used in combination with

phototherapy and chemotherapy, though pure chemotherapy is rarely used today.[3] No single treatment type has revealed clear-cut benefits in comparison to others, treatment for all cases remains problematic.[15]

Radiation therapy

A number of types of radiation therapy may be used including

total skin electron therapy.[16] While this therapy does not generally result in systemic toxic effects it can produce side effects involving the skin.[16] It is only available at a few institutions.[16]

Chemotherapy

Romidepsin, vorinostat and a few others are a second-line drug for cutaneous T-cell lymphoma.[17] Mogamulizumab has been approved in Japan[18] and the United States.[19]

Epidemiology

In the Western population, there are around 3 cases of Sézary syndrome per 1,000,000 people.[3] Sézary disease is more common in males with a ratio of 2:1,[3] and the mean age of diagnosis is between 55 and 60 years of age.[3][9]

See also

  • List of cutaneous conditions

References

  1. ^ Reference, Genetics Home. "Sézary syndrome". Genetics Home Reference.
  2. Who Named It?
  3. ^ a b c d e f g h i j k Cuneo, A; Castoldi, GL (2005). "Mycosis fungoidses/Sezary's syndrome". Atlas Genet Cytogenet Oncol Haematol. 9 (3): 242–243. Retrieved 2008-02-15.
  4. PMID 9129044
    .
  5. ISBN 978-1-4051-1376-2
    .
  6. ISBN 978-3-319-14728-4
    .
  7. ^ "Sezary syndrome". genetic and rare diseases information center. Retrieved 17 April 2018.
  8. PMID 28096719
    .
  9. ^ a b Lorincz, A. I. "Sezary syndrome". Retrieved 2008-02-15.
  10. PMID 29026585
    .
  11. S2CID 44779503
    .
  12. ^ a b c "Diagnosis". Retrieved 2008-02-15.
  13. PMID 13618707. {{cite book}}: |journal= ignored (help
    )
  14. ^ "Mycosis Fungoides (Including Sézary Syndrome) Treatment". National Cancer Institute. 7 September 2017. Retrieved 1 December 2017.
  15. ISBN 978-1-4051-1376-2
    .
  16. ^ a b c "Mycosis Fungoides (Including Sézary Syndrome) Treatment". National Cancer Institute. 12 July 2017. Retrieved 1 December 2017.
  17. PMID 24438970
    .
  18. PMID 22686619
    .
  19. ^ "FDA approves mogamulizumab-kpkc for mycosis fungoides or Sézary syndrome". U.S. Food and Drug Administration. 2018-08-08. Retrieved 2020-08-04.

External links

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