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Treatment of Sézary syndrome

Authors
Ellen J Kim, MD
Alain H Rook, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Sézary syndrome (SS) is a rare, aggressive subtype of cutaneous T cell lymphoma (CTCL), distinct from, but closely related to, mycosis fungoides (MF), that presents with exfoliative erythroderma and significant numbers of circulating malignant T cells (Sézary cells). SS can involve lymph nodes and visceral organs in some patients.

SS may either be considered to have evolved from MF (ie, not initially meeting the criteria for SS) or may present de novo with full blown signs and symptoms of the condition. In either case, both MF and SS are defined histologically and staged by the same criteria. Since 2007, SS has been defined as T4 (erythroderma) with B2 (eg, absolute Sézary cell count of at least 1000 cells/microL) blood involvement using the same TNMB classification syndrome as for MF (table 1A). In contrast to patch/plaque MF, SS is usually much more symptomatic, has a lower potential for remission, and lower expected survival.

The treatment of SS will be discussed here. The clinical presentation, diagnosis, and staging of SS are presented separately, as is the diagnosis and treatment of MF. (See "Clinical presentation, pathologic features, and diagnosis of Sézary syndrome" and "Staging and prognosis of mycosis fungoides and Sézary syndrome" and "Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides" and "Treatment of early stage (IA to IIA) mycosis fungoides" and "Treatment of advanced stage (IIB to IV) mycosis fungoides".)

STAGING

The standard staging system for mycosis fungoides (MF) and Sézary syndrome (SS) is based upon an evaluation of the skin (T), lymph nodes (N), visceral involvement (M), and blood (B). Details are presented separately (table 1A and table 1B). (See "Staging and prognosis of mycosis fungoides and Sézary syndrome", section on 'Staging'.)

SS is equivalent to the T4 plus B2 designation in the TNMB classification syndrome for MF. T4 erythrodermic skin disease characterizes those cases with erythema covering ≥80 percent body surface area. B2 involvement reflects the identification of a clonal T cell proliferation usually with an absolute Sézary cell count of at least 1000 cells/microL. (See "Clinical presentation, pathologic features, and diagnosis of Sézary syndrome", section on 'Diagnostic criteria'.)

                                     

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Literature review current through: Nov 2016. | This topic last updated: Thu Dec 17 00:00:00 GMT+00:00 2015.
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