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Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management
Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histop...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2023
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10140754/ https://www.ncbi.nlm.nih.gov/pubmed/37124514 http://dx.doi.org/10.3389/fonc.2023.1141108 |
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author | Miyashiro, Denis Sanches, José Antonio |
author_facet | Miyashiro, Denis Sanches, José Antonio |
author_sort | Miyashiro, Denis |
collection | PubMed |
description | Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS. |
format | Online Article Text |
id | pubmed-10140754 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-101407542023-04-29 Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management Miyashiro, Denis Sanches, José Antonio Front Oncol Oncology Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS. Frontiers Media S.A. 2023-04-14 /pmc/articles/PMC10140754/ /pubmed/37124514 http://dx.doi.org/10.3389/fonc.2023.1141108 Text en Copyright © 2023 Miyashiro and Sanches https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Oncology Miyashiro, Denis Sanches, José Antonio Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
title | Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
title_full | Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
title_fullStr | Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
title_full_unstemmed | Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
title_short | Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
title_sort | mycosis fungoides and sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management |
topic | Oncology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10140754/ https://www.ncbi.nlm.nih.gov/pubmed/37124514 http://dx.doi.org/10.3389/fonc.2023.1141108 |
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