Mycosis fungoides

Definition:

Mycosis fungoides (MF) is the most common type of primary cutaneous T-cell lymphomas (CTCL), which are a heterogeneous group of malignancies derived from skin-homing T cells. MF presents in the skin with erythematous patches, plaques, and less frequently, tumours. Although the aetiologies of MF are unknown, important insights have been gained in the immunological and genetic perturbations that are associated with these diseases. Mutations in the p53, p15, p16, JunB, and PTEN genes generally occur in later-stage disease. Loss of normal apoptotic T-cell pathways has also been reported. Apoptosis is partly mediated by death receptors, notably Fas, which is part of the tumor necrosis factor family of receptors. Decreased and/or defective Fas expression by neoplastic T cells has been associated with advanced/aggressive disease and impaired Fas-mediated apoptosis.

References:

[1]. Bruce R Smoller, et al. Mycosis fungoides: what do/do not we know?. J Cutan Pathol. 2008 Nov;35 Suppl 2:35-9. [Content Brief]

[2]. Elodie Laharanne, et al. CDKN2A-CDKN2B deletion defines an aggressive subset of cutaneous T-cell lymphoma. Mod Pathol. 2010 Apr;23(4):547-58. [Content Brief]

[3]. Leena Karenko, et al. Primary cutaneous T-cell lymphomas show a deletion or translocation affecting NAV3, the human UNC-53 homologue. Cancer Res. 2005 Sep 15;65(18):8101-10. [Content Brief]

[4]. Marc Beyer, et al. Pathogenesis of Mycosis fungoides. J Dtsch Dermatol Ges. 2011 Aug;9(8):594-8. [Content Brief]

[5]. P A Wu, et al. Interventions for mycosis fungoides: critical commentary on a Cochrane Systematic Review. Br J Dermatol. 2014 May;170(5):1015-20. [Content Brief]

[6]. Sam T Hwang, et al. Mycosis fungoides and Sézary syndrome. Lancet. 2008 Mar 15;371(9616):945-57. [Content Brief]

[7]. Sarah I Jawed, et al. Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): part I. Diagnosis: clinical and histopathologic features and new molecular and biologic markers. J Am Acad Dermatol. 2014 Feb;70(2):205.e1-16; quiz 221-2. [Content Brief]

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