REVIEW   

Giornale Italiano di Dermatologia e Venereologia 2017 April;152(2):158-68

DOI: 10.23736/S0392-0488.16.05527-9

Copyright © 2016 EDIZIONI MINERVA MEDICA

language: English

Latest insights into pathogenesis of mycosis fungoides and cutaneous T-cell lymphoma

Laura Y. MCGIRT ✉

Department of Hematology Oncology, Levine Cancer Institute, Charlotte, NC, USA

Cutaneous T-cell lymphoma (CTCL) is a rare but increasing malignancy whose protean manifestations necessarily present in the integument, but can also spread to involve blood, lymph nodes and internal organs. We have developed efficacious and varied therapies to treat early and advanced stage disease, but there are still many who suffer tremendously from this illness. Although the pathogenesis of this cancer remains frustratingly elusive, over the last 200 years we have generated a robust body of evidence that points toward possible singular as well as multifactorial etiologies. Combining the historical hypotheses which have focused upon the concept of infectious causes, including carcinogenic genomic viral integration and bacterial superantigenic chronic stimulation as well as industrial/occupational exposure, along with the more recent revelations of both genetic and epigenetic alteration and immune dysregulation, we are closer than ever to understanding the etiology of CTCL. It is through this knowledge and continued research efforts that we will be able to better diagnose, treat, and potentially prevent or cure CTCL.

KEY WORDS: Mycosis fungoides - Cutaneous T-cell lymphoma - Etiology