Diagnostic tools in Sezary syndrome.
Primary cutaneous T-cell lymphomas (CTCL) mycosis fungoides (Mf) and Sézary syndrome (SS) belong to the group of non-Hodgkin lymphomas which are characterized by clonally proliferating CD4+ cells localized in the skin. SS is a leukemic variant of CTCL and is characterized by erythroderma, generalized lymphadenopathy, and circulating atypical T-cells with cerebriform nuclei, so-called Sézary cells. Palmoplantar hyperkeratosis, generalized alopecia, and severe pruritus are additional symptoms that are associated with SS. Patients have a poor prognosis with an estimated five year survival of 12.5 to 27 percent and estimated median survival of 14.5 to 18 months. The incidence of MF and also SS has increased with time and may be in part due to improved clinical awareness and especially advances in diagnostic testing.
Möbs
M
M
Department of Dermatology and Allergy, Skin Cancer Center, Charité Universitätsmedizin, Berlin, Germany.
Knott
M
M
Fritzen
B
B
Pullmann
S
S
Sterry
W
W
Assaf
C
C
D015329:Gene Rearrangement, T-Lymphocyte; D006801:Humans; D012751:Sezary Syndrome / Q000235:genetics / Q000473:pathology*; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*