Mycosis fungoides.
Mycosis fungoides (MF) constitutes the most frequent cutaneous T-cell lymphoma. Sezary syndrome is considered by some authors to be an erythrodermic leukemic variant of MF, but is classified separately in the new WHO-EORT classification of cutaneous lymphomas. MF usually occurs in old adults with a 2:1 male to female ratio. Its prognosis is variable and strongly conditioned by the extent and type of skin involvement and presence of extracutaneous disease. Patients with stage IA-disease have an excellent prognosis with an overall long-term life expectancy that is similar to an age-, sex-, and race-matched control population. Almost all patients with stage IA MF will die from causes other than MF, with a median survival >33 years. Only 9% of these patients will progress to more extended disease. Patients with stage IB or IIA have a median survival greater than 11 years. These patients with T2 disease have a likelihood of disease progression of 24% and nearly 20% die of MF. Subgroups with stage IB or IIA have similar prognosis. Patients with cutaneous tumors or generalized erythroderma have a median survival of 3 and 4.5 years, respectively. The majority of these patients will die of MF. Extracutaneous dissemination is observed in less than 10% of patients with patch or plaque disease and in 30-40% of patients with tumors or generalized erythrodermatous involvement. Extracutaneous involvement is directly correlated to the extent of cutaneous disease. The most commonly involved organs are lung, spleen, liver, and gastrointestinal tract. Patients with extracutaneous disease at presentation involving either lymph nodes or viscera have a median survival of <1.5 years. Patients with plaque-type or erythrodermic MF may develop cutaneous tumors with large cell histology, often expressing CD30, which share a common clonal origin as observed in their preexisting MF and are associated with a less favourable outcome.
Zinzani
Pier Luigi
PL
L. and A. Seragnoli Institute of Hematology and Oncology, University of Bologna, Bologna, Italy. plzinzo@med.unibo.it
Ferreri
Andrés J M
AJ
Cerroni
Lorenzo
L
D006801:Humans; D009182:Mycosis Fungoides / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D009367:Neoplasm Staging; D011379:Prognosis; D012307:Risk Factors; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000628:therapy