TY - JOUR
T1 - Cutaneous T-Cell Lymphoma
T2 - Current and Emerging Therapies
AU - Dai, Julia
AU - Duvic, Madeleine
N1 - Publisher Copyright:
© 2023 UBM Medica Healthcare Publications. All rights reserved.
PY - 2023/2
Y1 - 2023/2
N2 - Cutaneous T-cell lymphomas (CTCLs) are clinically heterogeneous T-cell lymphomas that arise in the skin and are characterized by their clinical and pathological features. This review will focus on mycosis fungoides (MF) and Sézary syndrome (SS), which represent 60% to 80% and less than 10% of CTCL cases, respectively. While most patients with MF present with patches and plaques and can be successfully treated with skin-directed therapies, a minority of patients progress from early to advanced stages or undergo large cell transformation. SS is defined as erythroderma, lymphadenopathy, and more than 1000 circulating atypical T-cells/uL with cerebriform nuclei. It has a poor overall survival of 2.5 years. Given the overall rarity of CTCLs, it is notable that clinical trials of treatments for MF/SS have been successfully completed, resulting in FDA approvals of novel therapies with increasing overall response rates. This review outlines the current multidisciplinary approach to diagnosing and treating MF/SS, with a focus on combining skin-directed therapies with emerging targeted and investigational systemic therapies. Integrating these anticancer therapies with skin care and bacterial decolonization is critical for comprehensive management. Curing patients with MF/SS may be possible by using a personalized medicine approach including novel combination strategies, restoration of T helper 1 cytokines, and avoidance of immunosuppressive regimens.
AB - Cutaneous T-cell lymphomas (CTCLs) are clinically heterogeneous T-cell lymphomas that arise in the skin and are characterized by their clinical and pathological features. This review will focus on mycosis fungoides (MF) and Sézary syndrome (SS), which represent 60% to 80% and less than 10% of CTCL cases, respectively. While most patients with MF present with patches and plaques and can be successfully treated with skin-directed therapies, a minority of patients progress from early to advanced stages or undergo large cell transformation. SS is defined as erythroderma, lymphadenopathy, and more than 1000 circulating atypical T-cells/uL with cerebriform nuclei. It has a poor overall survival of 2.5 years. Given the overall rarity of CTCLs, it is notable that clinical trials of treatments for MF/SS have been successfully completed, resulting in FDA approvals of novel therapies with increasing overall response rates. This review outlines the current multidisciplinary approach to diagnosing and treating MF/SS, with a focus on combining skin-directed therapies with emerging targeted and investigational systemic therapies. Integrating these anticancer therapies with skin care and bacterial decolonization is critical for comprehensive management. Curing patients with MF/SS may be possible by using a personalized medicine approach including novel combination strategies, restoration of T helper 1 cytokines, and avoidance of immunosuppressive regimens.
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U2 - 10.46883/2023.25920984
DO - 10.46883/2023.25920984
M3 - Review article
C2 - 36862846
AN - SCOPUS:85149292362
SN - 0890-9091
VL - 37
SP - 55
EP - 62
JO - ONCOLOGY (United States)
JF - ONCOLOGY (United States)
IS - 2
ER -