Sézary cell: pleomorphic abnormal T cell with the characteristic cerebriform nuclei (Peripheral blood - MGG stain)
Sézary disease and
mycosis fungoides are
cutaneous T-cell lymphomas having a primary manifestation in the
skin.[5] The disease's origin is a peripheral CD4+ T-lymphocyte,[3] although rarer CD8+/CD4- cases have been observed.[3] Epidermotropism (lymphocytes residing in the epidermis)[6] by
neoplasticCD4+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.[7][3] The dominant signs and symptoms of the disease are:
Atypical
T cells – malignant lymphocytes known as "Sézary cells" seen in the peripheral blood with typical cerebriform nuclei (brain-shaped, convoluted nuclei)[8][3]
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]
More monotonous cellular infiltrates (large, clustered atypical
pagetoid cells) in Sézary syndrome
Sometimes absent epidermotropism
Increased lymph node involvement with infiltrates of Sézary syndrome.
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]
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]
^Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 39.
ISBN978-1-4051-1376-2.
^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.
ISSN1365-4632.
PMID22994666.
S2CID44779503.
^Cerroni, Lorenzo; Kevin Gatter; Helmut Kerl (2005). An illustrated guide to Skin Lymphomas. Malden, Massachusetts: Blackwell Publishing. p. 41.
ISBN978-1-4051-1376-2.
^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.
PMID24438970.