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Mycosis fungoides and Sézary syndrome.
| Content Provider | Europe PMC |
|---|---|
| Author | Lee, Hyewon |
| Copyright Year | 2023 |
| Abstract | Mycosis fungoides (MF) and Sézary syndrome (SS) are a distinct disease entity of cutaneous T-cell lymphoma with heterogenous clinical features and prognosis. MF mainly involves skin and usually shows an indolent and favorable clinical course. In patients with advanced-stage disease, extracutaneous involvement including lymph nodes, viscera, and blood, or large cell transformation may be observed. SS is a leukemic form of advanced-stage MF, characterized by generalized erythroderma. Early-stage MF can be treated with skin-directed therapy. However, patients with refractory or advanced-stage disease are associated with severe symptoms or poor prognosis, requiring systemic therapy. Recent progress in understanding the pathogenesis of MF/SS has contributed to advances in the management of these rare diseases. This review aims to describe the clinical manifestations, diagnosis, risk stratification, and treatment strategy of MF/SS, focusing on the recent updates in the management of these diseases. |
| Related Links | https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC10133849&blobtype=pdf |
| ISSN | 2287979X |
| Volume Number | 58 |
| DOI | 10.5045/br.2023.2023023 |
| PubMed Central reference number | PMC10133849 |
| Issue Number | Suppl 1 |
| Issue Number | s1 |
| PubMed reference number | 37105561 |
| Journal | Blood Research [Blood Res] |
| e-ISSN | 22880011 |
| Language | English |
| Publisher | Korean Society of Hematology |
| Publisher Date | 2023-04-01 |
| Access Restriction | Open |
| Rights License | This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2023 Korean Society of Hematology |
| Subject Keyword | Mycosis fungoides Sézary syndrome Cutaneous T-cell lymphoma Diagnosis Prognosis Treatment |
| Content Type | Text |
| Resource Type | Article |
| Subject | Hematology |
