Mycosis fungoides (MF) is the most common type of cutaneous T – cell lymphomas, accounting for for 50% of all cutaneous lymphomas. Its reported frequency is heterogeneous worldwide, with an incidence of ~ 0.58 cases per 100,000 person-years in the United States and 0.2–0.37 cases per 100,000 person-years across Europe; however, its incidence is thought to be greatly underestimated. They manifest heterogeneous clinical, histologic, immunophenotypic, and cytogenetic features. MF is associated with several professions which have high risk of exposing petrochemical, textile and metal industry, painting, woodworking, and carpentry.
Sezary syndrome (SS) and MF are closely related T-cell neoplasms, that are are considered separately on the basis of differences in the clinical features and cell of origin. Despite their differences, both conditions can be challenging to diagnose, particularly in the absence of clinical symptoms. Erythroderma, generalized lymphadenopathy, the presence of clonal neoplastic T cells with cerebriform nuclei (Sezary cells) in peripheral blood, skin and lymph nodes are the main features of the Sezary syndrome. Mycosis fungoides has an indolent course with slow progression over years characterized by patches, plaques and eventually tumours. Both SS and MF patients are adult/elderly and have male predominance.
Author(s) Details:
- C. Kariyawasan
Sri Jayewardenepura General Hospital, Thalapathpitiya Nugegoda, Sri Lanka. - L. T. Balasuriya
Sri Jayewardenepura General Hospital, Thalapathpitiya Nugegoda, Sri Lanka. - A. C. D. Ranatunga
Sri Jayewardenepura General Hospital, Thalapathpitiya Nugegoda, Sri Lanka.
Recent Global Research Developments in Understanding Mycosis Fungoides and Sézary Syndrome
Novel Agents and Therapies:
- Recent studies have highlighted the integration of novel agents into the treatment of advanced MF and SS. These include targeted therapies that have shown promise in inducing more durable responses and even complete remissions in some cases [1].
- For instance, agents like brentuximab vedotin and mogamulizumab have been approved for relapsed MF/SS, offering new hope for patients [2].
Skin-Directed and Immune-Based Treatments:
- Advances in skin-directed therapies include optimizing existing treatments and introducing new topical agents such as mechlorethamine gel and calcineurin inhibitor creams [2].
- Immune-based treatments are also being explored, with ongoing clinical trials investigating various cellular therapies and immunotherapies [2].
Molecular Biomarker-Guided Therapy:
- Research is increasingly focusing on molecular biomarkers to guide therapy, aiming to match treatments to patients most likely to benefit from them [1] .
Clinical Trials and Approvals:
- Numerous clinical trials are underway to evaluate the efficacy of new systemic agents, including monoclonal antibodies and Janus kinase (JAK) inhibitors [3] .
References
- Khodadoust, M. S., Mou, E., & Kim, Y. H. (2023). Integrating novel agents into the treatment of advanced mycosis fungoides and Sézary syndrome. Blood, 141(7), 695-703.
- Stuver R and Geller S (2023) Advances in the treatment of mycoses fungoides and Sézary syndrome: a narrative update in skin-directed therapies and immune-based treatments. Front. Immunol. 14:1284045. doi: 10.3389/fimmu.2023.1284045
- Quadri I, Reneau JC, Hanel W and Chung CG (2023) Advancements in the treatment of mycosis fungoides and Sézary syndrome: monoclonal antibodies, immunotherapies, and Janus kinase inhibitors. Front. Immunol. 14:1291259. doi: 10.3389/fimmu.2023.1291259
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