Clinical and Laboratory Features of Sezary Syndrome

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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