9701/3 Sezary syndrome


Synonyms

Sezary disease

Definitions

Tumours of haematopoietic and lymphoid tissues
ICD-O-3 topography code: C42 and C77
ICD10: C84.1
           

/span>?zary syndrome (SS) is defined by the triad of erythroderma, generalized lymphadenopathy and the presence of clonally related neoplastic T-cells with cerebriform nuclei (S?zary cells) in skin, lymph nodes and peripheral blood (PB). In addition, one or more of the following criteria are required: an absolute S?zary cell count of at least 1000 cells per mm3, an expanded CD4+ T-cell population resulting in a CD4/CD8 ratio of more than 10 and/or loss of one or more T-cell antigens.

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Skin
ICD-O-3 topography code: C44
ICD10: C84.1
           

A generalized peripheral (mature) T-cell neoplasm characterized by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sezary syndrome (SS) is an aggressive disease
2
 
Jaffe ES, Harris NL, Stein H, Vardiman JW (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues.
3rd Edition
IARC Press: Lyon 2001



3
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



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