51. Leukaemic variants of cutaneous T-cell lymphoma: Erythrodermic mycosis fungoides and Sézary syndrome.
- Author
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Martinez XU, Di Raimondo C, Abdulla FR, Zain J, Rosen ST, and Querfeld C
- Subjects
- Humans, Neoplasm Proteins immunology, Programmed Cell Death 1 Receptor immunology, Receptors, KIR3DL2 immunology, Immunologic Memory, Mycosis Fungoides diagnosis, Mycosis Fungoides immunology, Mycosis Fungoides pathology, Mycosis Fungoides therapy, Sezary Syndrome diagnosis, Sezary Syndrome immunology, Sezary Syndrome pathology, Sezary Syndrome therapy, Skin Neoplasms diagnosis, Skin Neoplasms immunology, Skin Neoplasms pathology, Skin Neoplasms therapy, T-Lymphocytes immunology, T-Lymphocytes pathology
- Abstract
Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common types of cutaneous lymphoma, accounting for approximately 60% of cutaneous T-cell lymphomas. Diagnosis requires correlation of clinical, histologic, and molecular features. A multitude of factors have been linked to the aetiopathogenesis, however, none have been definitively proven. Erythrodermic MF (E-MF) and SS share overlapping clinical features, such as erythroderma, but are differentiated on the degree of malignant blood involvement. While related, they are considered to be two distinct entities originating from different memory T cell subsets. Differential expression of PD-1 and KIR3DL2 may represent a tool for distinguishing MF and SS, as well as a means of monitoring treatment response. Treatment of E-MF/SS is guided by disease burden, patients' ages and comorbidities, and effect on quality of life. Current treatment options include biologic, targeted, immunologic, and investigational therapies that can provide long term response with minimal side effects. Currently, allogeneic stem cell transplantation is the only potential curative treatment., (Copyright © 2019. Published by Elsevier Ltd.)
- Published
- 2019
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