Abstract
SummaryCutaneous T cell lymphomas (CTCL) are a heterogeneous group of rare non-Hodgkin lymphomas. The most common type of CTCL is Mycosis fungoides (MF). Much less common but clinically and histopathologically related to MF is Sézary syndrome (SS). CTCL are incurable and associated with a reduced quality of life. While early stage MF has a good prognosis and is usually treated with skin directed therapies, advanced-stages require systemic therapies, including retinoids, interferon, cytotoxic chemotherapeutic drugs, low-dose methotrexate, histone deacetylase inhibitors and alemtuzumab. However, relapses are frequent and long-term remissions are achieved only in few cases, e.g. with allogenic stem cell transplantation. In recent years, new therapeutic options have evolved by the approval of brentuximab vedotin and mogamulizumab. Both recently approved therapies demonstrated superiority with regard to overall response rate and progression free survival over traditional systemic therapies. Other promising treatments such as lacutamab and PD-1/L-1 inhibitors are in the pipeline, and more therapeutic agents are currently investigated in clinical trials.
Highlights
Cutaneous T cell lymphomas (CTCL) are a heterogeneous group of rare non-Hodgkin lymphomas characterized by infiltration of neoplastic T lymphocytes to the skin
In CTCL CD30 expression is commonly seen in primary cutaneous anaplastic large-cell lymphoma and lymphomatoid papulosis [1] and less frequently in mycosis fungoides (MF) and Sézary syndrome (SS) at various levels, ranging from 20–40% [9,10,11]
A phase 2 study evaluating mogamulizuab in combination with total skin electron beam therapy (TSEBT) in CTCL patients is expected to enroll in May 2020 (MOGAT, NCT04128072) [18]
Summary
Summary Cutaneous T cell lymphomas (CTCL) are a heterogeneous group of rare non-Hodgkin lymphomas. The most common type of CTCL is Mycosis fungoides (MF). New therapeutic options have evolved by the approval of brentuximab vedotin and mogamulizumab. Both recently approved therapies demonstrated superiority with regard to overall response rate and progression free survival over traditional systemic therapies. Other promising treatments such as lacutamab and PD1/L-1 inhibitors are in the pipeline, and more therapeutic agents are currently investigated in clinical trials. Keywords Cutaneous T-cell lymphoma · Brentuximab vedotin · Mogamulizumab · Lacutamab · PD-1/L-1 inhibitors
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