Mycoisis fungoides and Sezary (CTCL) and stem cell transplantation – pro
Cutaneous T-cell lymphoma (CTCL) is classified as an indolent hematologic malignancy with distinct clinicopathologic features. Although prognosis varies depending on the stage, patients who have cutaneous tumor, lymph node or visceral involvement, or peripheral blood involvement (Sézary syndrome) generally have a poor outcome. Until recently, supporting literature was conflicting and of poor quality. However, a recent study from Sxhallak et al, found that allogeneic HCT in MF/SS results in 5-year survival in approximately one-third of patients and of those, half remain disease-free.
For advanced disease, systemic treatment options include low-dose methotrexate, photopheresis, biologic response modifiers such as bexarotene capsules, vorinostat (Zolinza), interferons, denileukin diftitox (Ontak), and single-agent chemotherapy. Combination therapies can be used when single agents fail or when patients have advanced or progressive disease.
Y. Oyama, J. Guitart, T. Kuzel, R. Burt, S. RosenHigh-dose therapy and bone marrow transplantation in cutaneous T-cell lymphoma.
Hematology/Oncology Clinics of North America, Volume 17, Issue 6, Pages 1475-1483, 2003.
Whittaker SJ, Marsden JR, Spittle M, Russell Jones R. Joint British Association of Dermatologists and U.K. Cutaneous Lymphoma Group guidelines for the management of primary cutaneous T-cell lymphomas. Br J Dermatol 2003 Dec;149(6):1095-107. [67 references
Treatment of advanced stage (IIB to IV) mycosis fungoides. Uptodate 2015
Authors
Richard T Hoppe, MD
Youn H Kim, MD
Steven Horwitz, MD
Section Editors
Timothy M Kuzel, MD
John A Zic, MD
Deputy Editors
Rebecca F Connor, MD
Rosamaria Corona, MD, DSc
M. Schallak et al, Aollogeneiic stem cell transplantation in patients with mycosis fungoides: results of a pooled analysis from 3 phase-II clinical trials. J Am Acad Dermatol 2015; 72:286.
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; 70:223.e1.