Sezary Syndrome is a T-cell leukemia/lymphoma in adults with skin and lymph node involvement. It tends to follow an aggressive course.
Clinical features:
(1) generalized erythroderma
(2) pruritis
(3) alopecia
(4) plantar and/or palmar hyperkeratosis
(5) onychodystrophy
(6) generalized lymphadenopathy
(7) visceral involvement in the terminal phases
Laboratory findings:
(1) atypical lymphoid infiltrate in the skin and lymph nodes
(2) circulating T cells with a convoluted (cerebriform) nucleus
(3) bone marrow often spared
Features of the atypical lymphocytes:
(1) CD2, CD3, CD4, CD5, CD7 positive (rarely CD8 positive)
(2) gene rearrangement in the T-cell receptor
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Specialty: Hematology Oncology, Dermatology
ICD-10: ,