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Clinical presentation, pathologic features, and diagnosis of Sézary syndrome

Alain H Rook, MD
Elise A Olsen, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD


Sézary syndrome (SS) and mycosis fungoides (MF) are the most common subtypes of cutaneous T cell lymphoma (CTCL) [1].

MF is a mature T cell non-Hodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera [2]. Skin lesions include patches, plaques, or tumors that may be localized or widespread and erythroderma.

SS is defined as a distinctive erythrodermic CTCL with a leukemic involvement of malignant T cells clonally matching that in the skin.

SS may be considered to have either 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 [1]. In either case, both MF and SS are defined histologically and staged by the same criteria [2]. In contrast to patch/plaque MF, SS is much more symptomatic, has a lower potential for remission, and lower expected survival.

In 2007, the staging system for MF and SS was revised to incorporate blood involvement and a standardized definition of erythroderma [2]. In 2011, further revisions to the blood and lymph node staging were published that further define and standardize the extracutaneous extent of the disease [3]. These changes in staging permit one to assess the outcomes in patients with erythrodermic MF separately from those of SS.  

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Literature review current through: Oct 2017. | This topic last updated: Jul 14, 2016.
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