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

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorMIYASHIRO, Denis
dc.contributor.authorSANCHES, Jose Antonio
dc.date.accessioned2023-06-21T14:07:44Z
dc.date.available2023-06-21T14:07:44Z
dc.date.issued2023
dc.description.abstractMycosis 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.eng
dc.description.indexPubMed
dc.description.indexWoS
dc.description.indexScopus
dc.identifier.citationFRONTIERS IN ONCOLOGY, v.13, article ID 1141108, 18p, 2023
dc.identifier.doi10.3389/fonc.2023.1141108
dc.identifier.issn2234-943X
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/53888
dc.language.isoeng
dc.publisherFRONTIERS MEDIA SAeng
dc.relation.ispartofFrontiers in Oncology
dc.rightsopenAccesseng
dc.rights.holderCopyright FRONTIERS MEDIA SAeng
dc.subjectmycosis fundgoideseng
dc.subjectSezary syndromeeng
dc.subjectcutaneous T cell lymphomaeng
dc.subjecthistopathologyeng
dc.subjectprognosiseng
dc.subjecttreatmenteng
dc.subject.othert-cell lymphomaeng
dc.subject.otherprimary cutaneous lymphomaseng
dc.subject.otherlong-term outcomeseng
dc.subject.otherprognostic-factorseng
dc.subject.othereuropean-organizationeng
dc.subject.otherunited-stateseng
dc.subject.otherclassification-systemeng
dc.subject.otherinternational-societyeng
dc.subject.otherclonal heterogeneityeng
dc.subject.othersingle-centereng
dc.subject.wosOncologyeng
dc.titleMycosis fungoides and Sezary syndrome: clinical presentation, diagnosis, staging, and therapeutic managementeng
dc.typearticleeng
dc.type.categoryrevieweng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.citation.scopus4
hcfmusp.contributor.author-fmusphcDENIS RICARDO MIYASHIRO
hcfmusp.contributor.author-fmusphcJOSE ANTONIO SANCHES JUNIOR
hcfmusp.description.articlenumber1141108
hcfmusp.description.volume13
hcfmusp.origemWOS
hcfmusp.origem.pubmed37124514
hcfmusp.origem.scopus2-s2.0-85159814797
hcfmusp.origem.wosWOS:000975961700001
hcfmusp.publisher.cityLAUSANNEeng
hcfmusp.publisher.countrySWITZERLANDeng
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