Mycosis Fungoides and Sezary Syndrome.
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.
Hematology and Bone Marrow Transplantation, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA. Electronic address: Francine.foss@yale.edu.
Francine M
FM
Foss
Department of Dermatology, Yale University School of Medicine, New Haven, CT 06510, USA.
Michael
M
Girardi
D064591:Allografts; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D009182:Mycosis Fungoides / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D012751:Sezary Syndrome / Q000378:metabolism / Q000473:pathology / Q000628:therapy*; D033581:Stem Cell Transplantation; D050378:T-Lymphocytes, Regulatory / Q000378:metabolism / Q000473:pathology
D007155:Immunologic Factors
Allogeneic stem cell transplantation; Immunotherapy; Mycosis fungoides; Sezary syndrome; T-cell lymphoma