Mycosis Fungoides

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Abstract
MYCOSIS fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL), with an estimated incidence of 0.5 per 100 000 per year in the western world.1 According to the major textbooks, MF is an indolent type of CTCL that slowly evolves through patch, plaque, and tumor stages before lymph nodes and visceral organs become involved, and ultimately a rapidly progressive and fatal disease develops. Long-term follow-up studies2-14 on large groups of patients with MF are rare, and most come from a few US-based centers. Comparison of published series is often difficult, because of different inclusion criteria (eg, inclusion or exclusion of patients with large-plaque parapsoriasis) and an inconsistent use of the terms MF and CTCL. Several studies, including one of the largest European studies of 92 Dutch patients published by Hamminga et al4 in 1982, included not only patients with classic MF but also patients with Sézary syndrome and other types of CTCL defined more recently. For instance, patients with CTCL presenting with tumors with the histological appearance of a diffuse, large, T-cell lymphoma and without prior or concurrent patches or plaques were designated previously as having "MF d'emblée," whereas such patients are now classified as having either CD30+ or CD30− large-cell CTCL, which are considered distinct disease entities separate from MF.15,16