Mycosis Fungoides and Sezary Syndrome.

Francine M Foss, Michael Girardi

Hematology/oncology clinics of North America 2017 Apr

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Mycosis fungoides and the Sezary syndrome (SS) are rare lymphomas of CD4+ helper T cells. There is stagewise progression from patch/plaques to thicker tumor lesions/diffuse erythroderma. Blood involvement is a characteristic of SS. Outcomes are related to the extent of skin, blood, lymph node, and visceral organ involvement. Patients with limited patch and plaque disease are treated with skin-directed therapies. More advanced/refractory disease is treated with skin-directed therapies and oral or systemic immunomodulatory agents. Single-agent chemotherapies are used against tumors, refractory plaques, and lymph node and visceral involvement. Allogeneic stem cell transplantation is a potentially curative strategy for advanced/resistant disease. Copyright © 2016 Elsevier Inc. All rights reserved.