Prognosis

Most cases of primary CTCL are not curable. Independent prognostic features in mycosis fungoides include the cutaneous and lymph node stage of disease and age of onset (>60 years). The lymph node status and tumour burden within peripheral blood determine the prognosis in Sezary syndrome.5,6 Serum lactate dehydrogenase and the thickness of the infiltrate in plaque-stage mycosis fungoides are also independent markers of prognosis.7 Multivariate analysis indicates that an initial complete response (CR) to various therapies is an independent favourable prognostic feature, particularly in early stages of disease.8-10 For mycosis fungoides, two staging systems are in regular use including a TNM (primary tumour, regional nodes, metastasis) system and a clinical staging specifically designed for CTCL (Box 27.1).5 These staging systems can also be applied to Sezary syndrome, but neither system provides a quantitative method for assessing peripheral blood disease other than an additional B0 and B1 in the TNM system and this has prompted alternative approaches for Sezary syndrome.6

Table 27.1 summarises recent actuarial survival data for mycosis fungoides. The 5- and 10-year overall survival (OS) in mycosis fungoides are 80% and 57%, respectively, with disease-specific survival (DSS) rates of 89% and 75% at 5 and 10 years respectively.8 Patients with very early stage disease (IA) are highly unlikely to die of their disease, with DSS rates of 100% and 97-98% at 5 and 10 years, respectively and risks of disease progression varying from 0% to 10% over 5-20 years.8-11 In one study of 122 patients with stage IA disease median survival was not reached at 32-5 years.9

Box 27.1 TNM (primary tumour, regional nodes, metastasis) classification for mycosis fungoides (including "B" system for cutaneous T-cell lymphoma (CTCL) to incorporate Sezary syndrome)

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