[Current therapeutic options in Mycosis fungoides and Sézary syndrome].

Driven by the approval of new targeted therapies, significant progress has been made in recent years in the clinical management of cutaneous T‑cell lymphomas. Although there are no curative treatment options for cutaneous T‑cell lymphomas, response rates are often encouraging, in particular when using combination therapies. The decision for the appropriate form of treatment depends on the specific diagnosis, disease stage, and the history of prior therapies. This article provides a comprehensive overview of current treatment options in mycosis fungoides and Sézary syndrome, based on the recently published, revised German S2k guidelines on cutaneous lymphomas (update 2021). In addition, we present promising, yet-to-be-approved therapies that at least in part can be already used off-label in clinical practice today.

Albrecht JD ，Nicolay JP 《-》

Mycosis fungoides and Sézary syndrome - Review and outlook.

Mycosis fungoides and Sézary syndrome are the most important representatives of the heterogeneous group of cutaneous T-cell lymphomas. The diseases are rare and the diagnosis, which always requires a clinical-pathological correlation, is often delayed, especially in early forms of mycosis fungoides. The prognosis of mycosis fungoides depends on its stage and is usually favorable in the early stages. Clinically relevant prognostic parameters are missing and their development is the subject of current clinical research. Sézary syndrome, characterized by initial erythroderma and blood involvement, is a disease with a high mortality rate, in which good responses can now be achieved in many cases with new treatment options. The pathogenesis and immunology of the diseases is heterogeneous, with recent results pointing primarily to changes in specific signal transduction pathways that may be suitable as future treatment targets. Current therapy for mycosis fungoides and Sézary syndrome is primarily palliative with topical and systemic options either used alone or in combination. Only with allogeneic stem cell transplantation durable remissions can be achieved in selected patients. Similar to other areas of oncology, the development of new therapies for cutaneous lymphomas is currently changing from relatively untargeted empiricism to disease-specific, targeted pharmacotherapy based on knowledge from experimental research.

Latzka J ，Trautinger F 《-》

[Cutaneous lymphomas : Clinical presentation - diagnosis - treatment].

Wobser M ，Goebeler M 《-》

[Cutaneous lymphomas : Clinical presentation - diagnosis - treatment].

Wobser M ，Goebeler M 《-》

Mycosis fungoides and Sézary syndrome: Australian clinical practice statement.

Primary cutaneous lymphomas represent a heterogeneous group of T- and B-cell lymphomas with distinct clinical presentations, histopathologic features, treatment approaches and outcomes. The cutaneous T-cell lymphomas, which include mycosis fungoides and Sézary syndrome, account for the majority of the cutaneous lymphomas. This Clinical Practice Statement is reflective of the current clinical practice in Australia. An expanded form of the Clinical Practice Statement (and updates), along with helpful patient resources and access to support groups, can be found at the following (http://www.australasianlymphomaalliance.org.au).

Bhabha FK ，McCormack C ，Wells J ，Campbell BA ，Newland K ，Lade S ，Buelens O ，Joske D ，Shortt J ，Mapp S ，Radeski D ，Hertzberg M ，Khot A ，Van Der Weyden C ，Khoo C ，Hawkes E ，Prince HM ... -  《-》