| Autor: |
Martínez Villarreal A; Division of Experimental Medicine, McGill University, Montreal, QC H4A3J1, Canada., Gantchev J; Division of Experimental Medicine, McGill University, Montreal, QC H4A3J1, Canada., Lagacé F; Division of Dermatology, McGill University, Montreal, QC H4A3J1, Canada., Barolet A; Division of Dermatology, McGill University, Montreal, QC H4A3J1, Canada., Sasseville D; Division of Dermatology, McGill University, Montreal, QC H4A3J1, Canada., Ødum N; LEO Foundation Skin Immunology Research Center, Department of Immunology and Microbiology, University of Copenhagen, DK-2200 Copenhagen, Denmark., Charli-Joseph YV; Cutaneous Hematopathology Clinic, Dermatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México 14080, Mexico.; Department of Dermatology, University of California San Francisco, San Francisco, CA 94115, USA., Hernández Salazar A; Cutaneous Hematopathology Clinic, Dermatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México 14080, Mexico., Litvinov IV; Division of Experimental Medicine, McGill University, Montreal, QC H4A3J1, Canada.; Division of Dermatology, McGill University, Montreal, QC H4A3J1, Canada. |
| Abstrakt: |
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. |
