Diagnostic tools in Sezary syndrome

Area of Science:

• Oncology
• Dermatology
• Hematology

Background:

• Primary cutaneous T-cell lymphomas (CTCL) include mycosis fungoides (MF) and Sézary syndrome (SS).
• Sézary syndrome (SS) is a leukemic variant of CTCL characterized by skin-localized, clonally proliferating CD4+ T-cells.
• SS presents with erythroderma, lymphadenopathy, circulating atypical T-cells, and severe symptoms like pruritus.

Purpose of the Study:

• To describe the characteristics and clinical presentation of Sézary syndrome (SS).
• To highlight the poor prognosis associated with SS.
• To discuss potential reasons for the increasing incidence of MF and SS.

Main Methods:

• Literature review and synthesis of existing data on CTCL, MF, and SS.
• Analysis of clinical features, diagnostic criteria, and survival statistics for SS.
• Examination of trends in incidence and potential contributing factors.

Main Results:

• SS is characterized by erythroderma, generalized lymphadenopathy, and circulating Sézary cells.
• Associated symptoms include palmoplantar hyperkeratosis, alopecia, and severe pruritus.
• Estimated five-year survival is 12.5-27%, with median survival of 14.5-18 months.

Conclusions:

• Sézary syndrome (SS) represents an aggressive form of CTCL with a significantly poor prognosis.
• Increased incidence may reflect enhanced diagnostic capabilities and heightened clinical awareness.
• Further research is warranted to improve understanding and treatment outcomes for SS.