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Hematol Oncol Clin North Am. 2017 Apr;31(2):297-315. doi: 10.1016/j.hoc.2016.11.008.

Mycosis Fungoides and Sezary Syndrome.

Author information

1
Hematology and Bone Marrow Transplantation, Yale University School of Medicine, 333 Cedar Street, New Haven, CT 06510, USA. Electronic address: Francine.foss@yale.edu.
2
Department of Dermatology, Yale University School of Medicine, New Haven, CT 06510, USA.

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

PMID:
28340880
DOI:
10.1016/j.hoc.2016.11.008
[Indexed for MEDLINE]
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