Mycosis Fungoides and Sezary Syndrome

Abstract

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.

Keywords: Allogeneic stem cell transplantation; Immunotherapy; Mycosis fungoides; Sezary syndrome; T-cell lymphoma.