Mycosis fungoides (MF) is an extranodal, indolent non-Hodgkin lymphoma of T cell origin that primarily develops in the skin, but can ultimately involve the lymph nodes, blood, and visceral organs. Early stage (IA to IIA) disease consists of papules, patches, or plaques, with limited, if any, lymph node involvement and no visceral involvement (table 1A-B). Patients with limited skin involvement plus the more aggressive histopathologic findings of folliculotropism or transformed large cell variants and those with blood involvement are treated as more advanced disease. (See 'Special scenarios' below.)
The management of early stage mycosis fungoides (MF) will be discussed here. The management of more advanced stage MF and the more aggressive leukemic variant, Sézary syndrome, is presented separately as are the clinical presentation, diagnosis, staging, and prognosis of MF and Sézary syndrome. (See "Treatment of advanced stage (IIB to IV) mycosis fungoides" and "Clinical manifestations, pathologic features, and diagnosis of mycosis fungoides" and "Treatment of Sézary syndrome".)
The standard staging system for mycosis fungoides (MF) is based upon an evaluation of the skin (T), lymph nodes (N), visceral involvement (M), and blood (B) (table 1A-B and table 2) . Details are presented separately. (See "Staging and prognosis of mycosis fungoides and Sézary syndrome", section on 'Staging'.)
STAGE IA DISEASE
Overview — Stage IA disease includes those patients with patches, plaques, or papules that involve less than 10 percent of the total skin surface with no involvement of lymph nodes or viscera (table 1A-B). Patients with stage IA disease who also have greater than 5 percent circulating atypical (Sézary) cells (ie, those with B1 disease), histologic evidence of the folliculotropic variant, or large cell transformed mycosis fungoides (MF) are treated with more aggressive therapy. (See 'Special scenarios' below.)
Patients with stage IA disease are treated with skin directed therapies. A randomized trial demonstrated that early aggressive therapy with combination chemotherapy plus electron-beam radiation therapy does not appear to improve survival when compared with the use of sequential topical regimens .