Mycosis Fungoides and Sezary Syndrome

Francine M Foss; Michael Girardi

doi:10.1016/j.hoc.2016.11.008

Mycosis Fungoides and Sezary Syndrome

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

Authors

Francine M Foss¹, Michael Girardi²

Affiliations

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.

PMID: 28340880
DOI: 10.1016/j.hoc.2016.11.008

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.

Publication types

Review

MeSH terms

Allografts
Humans
Immunologic Factors / therapeutic use*
Mycosis Fungoides / metabolism
Mycosis Fungoides / pathology
Mycosis Fungoides / therapy*
Sezary Syndrome / metabolism
Sezary Syndrome / pathology
Sezary Syndrome / therapy*
Stem Cell Transplantation*
T-Lymphocytes, Regulatory / metabolism
T-Lymphocytes, Regulatory / pathology

Substances

Immunologic Factors