Abstract
Hypopigmented mycosis fungoides (HMF) is a form of cutaneous T-cell lymphoma (CTCL), a heterogeneous group of extranodal non-Hodgkin’s lymphomas. HMF has a unique set of defining features that include light colored to achromic lesions, a predilection for darker skin phototypes, an early onset of disease, and predominance of CD8+ T-cells, among others. In the current review, we detail the known pathways of molecular pathogenesis for this lymphoma and posit that an active Th1/cytotoxic antitumor immune response in part explains why this variant is primarily seen in children/adolescents and young adults, who do not exhibit signs of immunosenescence. As a result of this potent cytotoxic response, HMF patients experience mostly favorable overall prognosis, while hypopigmentation may in fact represent a useful surrogate marker of cytotoxic immunity targeting the malignant cells. Understanding the molecular processes behind the specific features that define HMF may lead to improved diagnostic accuracy, personalized prognosis by risk stratification, and improved management of HMF. Moreover, improving our knowledge of HMF may aid our further understanding of other cutaneous lymphomas.
Highlights
Mycosis fungoides (MF) is a form of cutaneous T-cell lymphoma (CTCL), a heterogeneous group of extranodal non-Hodgkin’s lymphomas characterized by the expansion of monoclonal T-cells involving the skin [1,2,3]
The immunopathogenesis of MF implies an activation of the cytotoxic immune response, the adaptive antitumor immunity
We suggest that Hypopigmented mycosis fungoides (HMF) remains in the equilibrium phase, which is characterized by a balance between surviving and dying tumor cells
Summary
Mycosis fungoides (MF) is a form of cutaneous T-cell lymphoma (CTCL), a heterogeneous group of extranodal non-Hodgkin’s lymphomas characterized by the expansion of monoclonal T-cells involving the skin [1,2,3]. Conventional Alibert-Bazin MF of onset for HMF is much earlier, with several cases reported in pediatric, adolescent, and early adulthood commonly appears in older patients with a median age between 55 and 60 years at diagnosis [7]. Contrast, the age of onset for HMF is much earlier, with several cases reported in pediatric, adolescent, HMF has a better prognosis than the conventional MF. The disease has been reported in children as young as 6 months of best prognostic factors for conventional Alibert-Bazin MF [1], which are determined by the skin disease age [19]. HMF, in general, has an excellent prognosis and the majority of Cancers 2020, 12, 2007 patients are diagnosed at an early stage (IA–IB) of the disease They present with an indolent form and they rarely progress beyond stage IB [8]. A striking epidermotropism and a predominance of clonal malignant CD8+ T-cells are the two common histologic features of HMF, which other MF variants do not exhibit as often [6,8]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
