Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): A practical compartment-based review.

Primary cutaneous lymphomas are a heterogeneous group of lymphoid neoplasms with predominant skin involvement, among which mycosis fungoides (MF) and Sézary syndrome (SS) are the most frequent and clinically challenging entities. This narrative review provides a multidisciplinary, compartment-oriented approach to the diagnosis, staging and management of MF/SS. We summarize the therapeutic principles across the disease continuum, from early-stage, skin-directed management to advanced-stage systemic therapy, including oral retinoids, methotrexate, extracorporeal photopheresis, mogamulizumab, brentuximab vedotin, chemotherapy and allogeneic stem-cell transplantation. We also address large-cell transformation, response assessment, follow-up strategies, and practical issues surrounding treatment maintenance, spacing and discontinuation in a chronic, relapsing disease. Finally, we highlight the importance of supportive care, particularly pruritus control and palliative integration when no further effective options remain, and provide pragmatic algorithms to facilitate real-world clinical decision-making.
Cancer
Care/Management

Authors

Mozas Mozas, Combalia Combalia, Antelo Antelo, L贸pez-Oreja L贸pez-Oreja, Castrej贸n de Anta Castrej贸n de Anta, Monge-Escart铆n Monge-Escart铆n, Moreno-Alonso Moreno-Alonso, Gil-Lianes Gil-Lianes, Juncosa Juncosa, Salas Salas, Charry Charry, Albero Albero, Garc铆a-Herrera Garc铆a-Herrera, L贸pez Guillermo L贸pez Guillermo, Estrach Estrach
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