Sezary syndrome


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Related to Sezary syndrome: mycosis fungoides

Sézary syndrome

 [sa´zah-re]
a form of cutaneous T-cell lymphoma manifested by generalized exfoliative erythroderma, intense pruritus, peripheral lymphadenopathy, and abnormal hyperchromatic mononuclear cells in the skin, lymph nodes, and peripheral blood (Sézary cells).

Sé·za·ry syn·drome

(sā-zah-rē'),
exfoliative dermatitis with intense pruritus, resulting from cutaneous infiltration by atypical mononuclear cells (T lymphocytes with markedly convoluted or cerebriform nuclei) also found in the peripheral blood, and associated with alopecia, edema, and nail and pigmentary changes; a variant of mycosis fungoides.

Sé·za·ry syn·drome

(sā-zah-rē'),
exfoliative dermatitis with intense pruritus, resulting from cutaneous infiltration by atypical mononuclear cells (T lymphocytes with markedly convoluted or cerebriform nuclei) also found in the peripheral blood, and associated with alopecia, edema, and nail and pigmentary changes; a variant of mycosis fungoides.

Sezary syndrome

Mycosis fungoides, see there.

Sé·za·ry syn·drome

(sā-zah-rē' sindrōm)
Exfoliative dermatitis with intense pruritus, resulting from cutaneous infiltration by atypical mononuclear cells also found in the peripheral blood, and associated with alopecia, edema, and nail and pigmentary changes; a variant of mycosis fungoides.

Sezary syndrome

A rare condition featuring a malignant spread of excessive numbers of abnormal T LYMPHOCYTES in the blood, with enlarged lymph nodes and deposits in the skin, liver and spleen. There are red, scaly patches on the skin extending to form a widespread, itchy and flaking rash, hair loss and distortion of the nails. The condition is thought to be caused by a retrovirus, HTLV-I. Treatment is with anticancer drugs and RADIOTHERAPY. (Albert Sezary, 1880–1956, French physician).

Sézary syndrome

A leukemic phase of CTCL that develops in some patients, characterized by the appearance of malignant T cells in the peripheral blood and sometimes in the lymph nodes. The syndrome is named for Alfred Sézary (1880–1956), a French dermatologist.

Bouvrain,

Y., French physician.
Sézary-Bouvrain syndrome - Synonym(s): Sézary syndrome

Sézary,

Albert, French dermatologist, 1880-1956.
Sézary-Bouvrain syndrome - Synonym(s): Sézary syndrome
Sézary cell - an atypical T lymphocyte seen in the peripheral blood in Sézary syndrome.
Sézary erythroderma - Synonym(s): Sézary syndrome; Sézary-Bovrain syndrome
Sézary syndrome - a variant of mycosis fungoides. Synonym(s): Sézary-Bouvrain syndrome; Sézary erythroderma
References in periodicals archive ?
Ralfkiaer et al., "Malignant Tregs express low molecular splice forms of FOXP3 in Sezary syndrome," Leukemia, vol.
Extracorporeal photopheresis in Sezary syndrome: hematologic parameters as predictors of response.
Lymphoid Neoplasms With Secondary Eosinophilia Lymphocyte-variant hypereosinophilia Cutaneous T-cell lymphoma, especially advanced-stage MF and SS Lymphoblastic leukemia/lymphoma, especially B ALL with t(5; 14)(q31; q32) Classical Hodgkin lymphoma Adult T-cell leukemia/lymphoma Angioimmunoblastic T-cell lymphoma Abbreviations: B ALL, B lymphoblastic leukemia; MF, mycosis fungoides; SS, Sezary syndrome. Table 6.
In the case of our patient, the diagnosis of Sezary syndrome was excluded because laboratory tests did not confirm the pres-ence of Sezary cells in the peripheral blood smear, only lympho-cytes with a similar morphology, whose numbers were continu-ally oscillating.
Prognostic factors and evaluation of mycosis fungoides and Sezary syndrome. J Am Acad Dermatol.
Sezary syndrome is a rare disease characterized by erythroderma, lymphadenopathy, and a neoplastic T-cell population involving skin, lymph nodes, and peripheral blood.
Cutaneous T-cell lymphoma is a heterogeneous class of non-Hodgkin's lymphoma, which is a type of cancer of the immune system, with the most common forms being mycosis fungoides (MF) and Sezary syndrome (SS).
The concept of primary cutaneous [CD4.sup.+] small/medium T-cell lymphoma (PC-SMTCL) began in the early 1990s when the updated Kiel classification system (1,2) was applied to primary cutaneous T-cell lymphomas that did not meet criteria for mycosis fungoides (MF) or Sezary syndrome. (3,4) These early studies established that lymphoid infiltrates with predominantly small, pleomorphic T-cells had a more favorable prognosis than those with predominantly large, pleomorphic T-cells.
[ClickPress, Tue Jan 07 2014] GlobalData's clinical trial report, "Sezary Syndrome Global Clinical Trials Review, H2, 2013" provides data on the Sezary Syndrome clinical trial scenario.
Among these cases, 23 tumors (74%) were T-cell and NK/T-cell lymphomas, including 5 NKTCLs (16%); 5 primary cutaneous peripheral T-cell lymphomas, unspecified (16%); 4 mycosis fungoides (MF; 13%), 3 primary cutaneous anaplastic large cell lymphomas (10%), 2 primary cutaneous small-medium [CD4.sup.+] T-cell lymphomas (6%), 2 lymphomatoid papulosis (6%), 1 Sezary syndrome (3%), and 1 subcutaneous panniculitislike T-cell lymphoma (3%); 8 cases (26%) were primary cutaneous B-cell lymphomas, including 3 primary cutaneous marginal zone B-cell lymphomas (10%), 3 primary cutaneous follicle center lymphomas (10%), and 2 primary cutaneous diffuse large B-cell lymphomas (6%), leg type.