Aetiology

The underlying aetiology is unknown. There is evidence for inactivation of key tumour suppressor genes and TH2 cytokine production by tumour cells in mycosis fungoides/Sezary syndrome, but no disease-specific molecular abnormality has yet been identified.4 Primary CTCL must be distinguished from human T-lymphotropic virus type-1 (HTLV-I) associated adult T-cell leukaemia lymphoma (ATLL) in which skin involvement often closely mimics the clinicopathological features of mycosis fungoides/Sezary syndrome and may be the presenting feature.1

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