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1688 Part XI: Malignant Lymphoid Diseases Chapter 103: Cutaneous T-Cell Lymphoma (Mycosis Fungoides and Sézary Syndrome) 1689

required. In rare cases, LyP evolves into more aggressive primary 21. Burg G, Dummer R, Haeffner A, et al: From inflammation to neoplasia: Mycosis fun-
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cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome). Br J Haematol
LyP is extremely responsive to low-dose methotrexate therapy, requir- 155:150–166, 2011.
ing 10 to 15 mg orally weekly, with noticeable clinical response within 25. Yawalkar N, Ferenczi K, Jones DA, et al: Profound loss of T-cell receptor repertoire
a month. Brentuximab vedotin was shown to be highly effective in complexity in cutaneous T-cell lymphoma. Blood 102:4059–4066, 2003.
patients with PCALCL and LyP (see section Monoclonal Antibody 26. Smoller BR: Risk of secondary cutaneous malignancies in patients with long-standing
mycosis fungoides. J Am Acad Dermatol 31:295, 1994.
Conjugates above). Other treatment options include oral PUVA ther- 27. Morales-Suarez-Varela MM, Olsen J, Johansen P, et al: Occupational risk factors for
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cell lymphoma depends on the extent of skin involvement. In cases of 28. Moreau JF, Buchanich JM, Geskin JZ, et al: Non-random geographic distribution of
patients with cutaneous T-cell lymphoma in the Greater Pittsburgh Area. Dermatol
solitary lesions, radiotherapy is often the best initial treatment modality. Online J 20, 2014.
A combination of PUVA and interferon-α may be considered for more 29. Talpur R, Singh L, Daulat S, et al: Long-term outcomes of 1,263 patients with mycosis
disseminated disease. Combination chemotherapy should be reserved fungoides and Sézary syndrome from 1982 to 2009. Clin Cancer Res 18:5051–5060,
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for resistant cases. 81,147,156 30. Vij A, Duvic M: Prevalence and severity of pruritus in cutaneous T cell lymphoma. Int
J Dermatol 51:930–934, 2012.
31. Nguyen V, Huggins RH, Lertsburapa T, et al: Cutaneous T-cell lymphoma and Staphy-
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