PMID: 9194581May 1, 1997Paper

Cutaneous histopathology of Sézary syndrome: a study of 41 cases with a proven circulating T-cell clone

Journal of Cutaneous Pathology
M J TrotterN P Smith

Abstract

Sezary syndrome is an uncommon variant of cutaneous T-cell lymphoma (CTCL) characterized by erythroderma, pruritus, adenopathy, and circulating atypical T-lymphocytes with cerebriform nuclei. The definition of Sezary syndrome can be further refined by including only patients with a circulating peripheral blood population of clonal T-cells. We have evaluated 79 skin biopsies from such a group of 41 erythrodermic patients with circulating Sezary cells and a clonal population of T-cells detected by T-cell receptor-beta gene rearrangement on Southern analysis of peripheral blood mononuclear cells. Histopathologic features consistent with chronic dermatitis were observed in 26/79 (33%) skin biopsy specimens, emphasizing that a non-specific histologic appearance is common. Evidence of CTCL was lacking in 11/41 patients on biopsy of their erythrodermic skin. The survival of these patients was not significantly different from 30/41 patients in whom skin biopsies revealed changes diagnostic of CTCL, such as a dermal lymphocytic band with atypical lymphocytes (18/79, 23%) or a mycosis fungoides-like infiltrate (30/79, 38%). This study confirms that non-specific cutaneous histopathologic findings are common in Sezary syndrome, even when a...Continue Reading

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Citations

Jun 19, 2010·The American Journal of Dermatopathology·Caroline Ram-WolffNicolas Ortonne
Aug 20, 2011·Seminars in Diagnostic Pathology·Alessandro PileriStefano A Pileri
Feb 24, 2009·Journal of the American Academy of Dermatology·Ginette A Hinds, Peter Heald
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Dec 17, 2014·Expert Review of Hematology·Bláithín Moriarty, Sean Whittaker

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