Mycosis fungoides and Sézary syndrome
| Autor: | Julia Tittes, Constanze Jonak, Patrick M. Brunner, Emmanuella Guenova |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Mycosis fungoides
Skin Neoplasms business.industry Cutaneous T-cell lymphoma Erythroderma Dermatology Disease medicine.disease Humans Lymphoma T-Cell Cutaneous Mycosis Fungoides/diagnosis Mycosis Fungoides/therapy Quality of Life Sezary Syndrome/diagnosis Sezary Syndrome/therapy Skin Neoplasms/diagnosis Skin Neoplasms/therapy Sézary syndrome cutaneous T-cell lymphoma dermatology mycosis fungoides Pathogenesis Transplantation Mycosis Fungoides Pathognomonic Immunology medicine Sezary Syndrome business Sezary Cell |
| Zdroj: | Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology, vol. 19, no. 9, pp. 1307-1334 |
| ISSN: | 1610-0387 |
| Popis: | Mycosis fungoides (MF) and Sezary syndrome (SS) are primary cutaneous T-cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor-cell burden (Sezary cells) in the peripheral blood consistent with advanced-stage disease and a poor prognosis. In early-stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early-stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin-directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets. |
| Databáze: | OpenAIRE |
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