Mycosis fungoides: Review of Epidemiological Observations

Abstract

Background: Mycosis fungoides (MF) is a chronic cutaneous T-cell lymphoma characterized by small cells with cerebriform nuclei that usually express a mature peripheral T-helper cell (CD4+) immunophenotype. Its evolution is typically quite slow, with years between the first manifestations and development of advanced stages of disease. Objective: The purpose of the present paper is to contribute to the material about MF already present in the literature. The review articles that have appeared to date fundamentally address the morphological characteristics, diagnostic criteria and treatment of the disease; in contrast, the present study centers on the evolution of the incidence of MF and on the knowledge of the possible risk factors implicated in its development. Methods: Review of published papers about MF epidemiology. Results: The evidence suggests that the incidence is increasing, but this may be artifactual due to improved diagnostic techniques. The risk of MF is limited to gender and race, being higher in males and in blacks. Survival is highly stage dependent, but 90% of patients survive 15 years with only 10% of cutaneous involvement. Few risk factors have been identified, but several studies have found an association with industrial exposure, particularly to oils. Conclusion: MF is a rare disease and its risk factors have not been studied in any great detail. A European case-control study in progress will substantially increase the evidence available and progress towards identifying a prevention strategy.

Cutaneous lymphoma incidence patterns in the United States: a population-based study of 3884 cases

“…Blacks had statistically higher IR of CTCL and MF than other races, consistent with previous studies. 4,26 To our knowledge, for the first time we reported that the CTCL IRs among Asian/ Pacific Islanders and Hispanic whites were lower than among blacks and non-Hispanic whites. In contrast to MF, IRs for primary cutaneous peripheral T-cell lymphoma and cutaneous CD30 ϩ T-cell lymphoproliferative disease were similar among blacks and nonHispanic whites.…”

Lesional gene expression profiling in cutaneous T-cell lymphoma reveals natural clusters associated with disease outcome

“…At this time, it is impossible to predict which patients with early-stage CTCL will progress, and which will continue to manifest indolent skin-limited disease. 9 Transcriptional analysis of cancer has proven to be a powerful and increasingly useful tool in biomedical research. [10][11][12] These patterns of gene expression have revealed unappreciated complexity in histologically homogeneous diseases like large-cell B-cell lymphoma.…”

Unusual involvement in mycosis fungoides: Duodenal papilla

“…The MF incidence is about 3 per million a year, affecting people between 55 to 60 years of age (2). The disease may have a slow, indolent evolution and causes skin lesions that include nodules, plaques, and tumors that can progress to compromise an extensive area of skin (3).…”

“…It is a rare disease, of low incidence, and usually affects middle-aged men (2). In most cases, only the skin is involved (3), although in advanced stages it may present with extracutaneous involvement, including the gastrointestinal tract (4,5).…”