Mycosis fungoides and Sezary syndrome: clinical presentation, diagnosis, staging, and therapeutic management

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art_MIYASHIRO_Mycosis_fungoides_and_Sezary_syndrome_clinical_presentation_diagnosis_2023.PDF (9.6 MB)
Citações na Scopus
3
Tipo de produção
article
Data de publicação
2023
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
FRONTIERS MEDIA SA
Autores
Citação
FRONTIERS IN ONCOLOGY, v.13, article ID 1141108, 18p, 2023
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Mycosis fungoides (MF) and Sezary 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.
Palavras-chave
mycosis fundgoides, Sezary syndrome, cutaneous T cell lymphoma, histopathology, prognosis, treatment
URI
https://observatorio.fm.usp.br/handle/OPI/53888
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