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Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions.
J Am Acad Dermatol 2014; 70(2):223.e1-17; quiz 240-2JA

Abstract

Both mycosis fungoides (MF) and Sézary syndrome (SS) have a chronic, relapsing course, with patients frequently undergoing multiple, consecutive therapies. Treatment is aimed at the clearance of skin disease, the minimization of recurrence, the prevention of disease progression, and the preservation of quality of life. Other important considerations are symptom severity, including pruritus and patient age/comorbidities. In general, for limited patch and plaque disease, patients have excellent prognosis on ≥1 topical formulations, including topical corticosteroids and nitrogen mustard, with widespread patch/plaque disease often requiring phototherapy. In refractory early stage MF, transformed MF, and folliculotropic MF, a combination of skin-directed therapy plus low-dose immunomodulators (eg, interferon or bexarotene) may be effective. Patients with advanced and erythrodermic MF/SS can have profound immunosuppression, with treatments targeting tumor cells aimed for immune reconstitution. Biologic agents or targeted therapies either alone or in combination--including immunomodulators and histone-deacetylase inhibitors--are tried first, with more immunosuppressive therapies, such as alemtuzumab or chemotherapy, being generally reserved for refractory or rapidly progressive disease or extensive lymph node and metastatic involvement. Recently, an increased understanding of the pathogenesis of MF and SS with identification of important molecular markers has led to the development of new targeted therapies that are currently being explored in clinical trials in advanced MF and SS.

Authors+Show Affiliations

Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Lymphoma Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York.Dermatology Service, Department of Medicine, Memorial Sloan-Kettering Cancer Center and Weill Cornell Medical College, New York, New York. Electronic address: querfelc@mskcc.org.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

24438970

Citation

Jawed, Sarah I., et al. "Primary Cutaneous T-cell Lymphoma (mycosis Fungoides and Sézary Syndrome): Part II. Prognosis, Management, and Future Directions." Journal of the American Academy of Dermatology, vol. 70, no. 2, 2014, pp. 223.e1-17; quiz 240-2.
Jawed SI, Myskowski PL, Horwitz S, et al. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions. J Am Acad Dermatol. 2014;70(2):223.e1-17; quiz 240-2.
Jawed, S. I., Myskowski, P. L., Horwitz, S., Moskowitz, A., & Querfeld, C. (2014). Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions. Journal of the American Academy of Dermatology, 70(2), pp. 223.e1-17; quiz 240-2. doi:10.1016/j.jaad.2013.08.033.
Jawed SI, et al. Primary Cutaneous T-cell Lymphoma (mycosis Fungoides and Sézary Syndrome): Part II. Prognosis, Management, and Future Directions. J Am Acad Dermatol. 2014;70(2):223.e1-17; quiz 240-2. PubMed PMID: 24438970.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part II. Prognosis, management, and future directions. AU - Jawed,Sarah I, AU - Myskowski,Patricia L, AU - Horwitz,Steven, AU - Moskowitz,Alison, AU - Querfeld,Christiane, PY - 2013/07/26/received PY - 2013/08/13/revised PY - 2013/08/16/accepted PY - 2014/1/21/entrez PY - 2014/1/21/pubmed PY - 2014/3/19/medline KW - BSA KW - CR KW - CRR KW - CTCL KW - ECP KW - EORTC KW - European Organization of Research and Treatment of Cancer KW - HDACi KW - IFNα KW - ISCL KW - International Society for Cutaneous Lymphoma KW - MF KW - NBUVB KW - NCCN KW - NK KW - NM KW - NMSC KW - National Comprehensive Cancer Network KW - ORR KW - PUVA KW - RAR KW - RXR KW - SS KW - Sézary syndrome KW - TNMB KW - TSEBT KW - USCLC KW - UVB KW - United States Cutaneous Lymphoma Consortium KW - body surface area KW - complete response KW - complete response rate KW - cutaneous T-cell lymphoma KW - extracorporeal photopheresis KW - histone deacetylase inhibitor KW - immunomodulators KW - interferon-alfa KW - mSWAT KW - modified severity-weighted assessment tool KW - mycosis fungoides KW - narrowband ultraviolet B light KW - natural killer KW - nitrogen mustard KW - nonmelanoma skin cancer KW - overall response rate KW - phototherapy KW - prognosis KW - psoralen plus ultraviolet A light phototherapy KW - retinoic acid receptor KW - retinoid X receptor KW - skin-directed treatment KW - staging KW - systemic treatment KW - targeted therapies KW - topical nitrogen mustard KW - topical retinoids/rexinoids KW - topical corticosteroids KW - total skin electron beam therapy KW - tumor, node, metastasis, blood KW - ultraviolet B light SP - 223.e1-17; quiz 240-2 JF - Journal of the American Academy of Dermatology JO - J. Am. Acad. Dermatol. VL - 70 IS - 2 N2 - Both mycosis fungoides (MF) and Sézary syndrome (SS) have a chronic, relapsing course, with patients frequently undergoing multiple, consecutive therapies. Treatment is aimed at the clearance of skin disease, the minimization of recurrence, the prevention of disease progression, and the preservation of quality of life. Other important considerations are symptom severity, including pruritus and patient age/comorbidities. In general, for limited patch and plaque disease, patients have excellent prognosis on ≥1 topical formulations, including topical corticosteroids and nitrogen mustard, with widespread patch/plaque disease often requiring phototherapy. In refractory early stage MF, transformed MF, and folliculotropic MF, a combination of skin-directed therapy plus low-dose immunomodulators (eg, interferon or bexarotene) may be effective. Patients with advanced and erythrodermic MF/SS can have profound immunosuppression, with treatments targeting tumor cells aimed for immune reconstitution. Biologic agents or targeted therapies either alone or in combination--including immunomodulators and histone-deacetylase inhibitors--are tried first, with more immunosuppressive therapies, such as alemtuzumab or chemotherapy, being generally reserved for refractory or rapidly progressive disease or extensive lymph node and metastatic involvement. Recently, an increased understanding of the pathogenesis of MF and SS with identification of important molecular markers has led to the development of new targeted therapies that are currently being explored in clinical trials in advanced MF and SS. SN - 1097-6787 UR - https://www.unboundmedicine.com/medline/citation/24438970/Primary_cutaneous_T_cell_lymphoma__mycosis_fungoides_and_Sézary_syndrome_:_part_II__Prognosis_management_and_future_directions_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190-9622(13)00907-9 DB - PRIME DP - Unbound Medicine ER -