Cutaneous T-cell Lymphomas: Mycosis Fungoides and Sézary Syndrome. Diagnostics and Therapy

Česká verzia

Authors: P. Fialová ¹
Authors place of work: I. dermatovenerologická klinika FN u sv. Anny v Brně a LF MU, přednosta prof. MUDr. Hana Jedličková, Ph. D. ¹
Published in the journal: Čes-slov Derm, 97, 2022, No. 2, p. 45-59
Category: Reviews (Continuing Medical Education)

Summary

Primary cutaneous lymphomas (PCL) form one autonomous and heterogenous group of extranodal non-Hodgkin lymphomas, which typically present malignant T-cells or B-cells in the skin without extracutaneous disease at the time of diagnosis. The WHO-EORTC classification divides PCL into cutaneous T-cell lymphomas (CTCL), which represent about 75% of cases and cutaneous B-cell lymphomas (CBCL). Mycosis fungoides (MF) and Sézary syndrome (SS) together represent the most frequent subtype of CTCL, which is about one third of all cases of CTCL. Mycosis fungoides is a long-term indolent tumour disease developing typically in three stages – patches, plaques and tumours. Sézary syndrome is a rare disease with aggressive course characterized by erythroderma, generalized lymphadenopathy and presence of cutaneous T-cells in blood. Clinical picture, histological, immunohistochemical and genetic examination are essential for the diagnosis of MF/SS. CTCL are recently incurable. The aim of the therapy is to prolong survival with good quality of life. Therapy is divided into skin directed and systemic. Patients with CTCL require multidisciplinary treatment in specialized centres with expertise in the management of cutaneous lymphomas.

Keywords:

staging – Prognosis – mycosis fungoides – diagnostics – systemic treatment – Sézary syndrome – cutaneous T-cell lymphomas – skin directed therapy

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