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Treatment of advanced stage (IIB to IV) mycosis fungoides

Authors
Richard T Hoppe, MD
Youn H Kim, MD
Steven Horwitz, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editors
Alan G Rosmarin, MD
Rosamaria Corona, MD, DSc

INTRODUCTION

Mycosis fungoides (MF) is an extranodal, usually indolent non-Hodgkin lymphoma of T cell origin that primarily develops in the skin, but can ultimately involve the lymph nodes, blood, and visceral organs. Sézary syndrome (SS) is a more aggressive leukemic variant of cutaneous T cell lymphoma (CTCL) in which circulating malignant (Sézary) cells are observed in the peripheral blood.

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

PRETREATMENT EVALUATION

The standard staging system for MF is based upon an evaluation of the skin (T), lymph nodes (N), visceral involvement (M), and blood (B) (table 1A-B and table 2) [1]. Details are presented separately. (See "Staging and prognosis of mycosis fungoides and Sézary syndrome", section on 'Staging'.)

GOALS OF THERAPY

Advanced stage MF is most often a chronic disease with a persistent or relapsing course. Management is often orchestrated by a multidisciplinary team comprised of dermatologists, medical oncologists, and radiation oncologists. The choice of therapy at different time points is largely dependent upon the goals of therapy, which include:

Long term disease control – Long term disease control is the main goal of therapy for most patients with MF. Since most agents have a short duration of response after the cessation of therapy, maintenance therapy that incorporates agents with low toxicity, an absence of cumulative toxicity, and preservation of the immune response is usually preferred, even if these agents have a slower time to response or lower overall response rate than more toxic drugs. (See 'Long-term disease control' below.)

                            

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Literature review current through: Nov 2016. | This topic last updated: Tue Feb 09 00:00:00 GMT+00:00 2016.
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