TY - JOUR

T1 - Hypopigmented mycosis fungoides

T2 - Loss of pigmentation reflects antitumor immune response in young patients

AU - Villarreal, Amelia Martínez

AU - Gantchev, Jennifer

AU - Lagacé, François

AU - Barolet, Augustin

AU - Sasseville, Denis

AU - Ødum, Niels

AU - Charli-Joseph, Yann Vincent

AU - Salazar, Amparo Hernández

AU - Litvinov, Ivan V.

PY - 2020

Y1 - 2020

N2 - 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.

AB - 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.

KW - Antitumor immune response

KW - Cutaneous T-cell lymphomas

KW - Cytotoxic cells

KW - Hypopigmentation

KW - Hypopigmented mycosis fungoides

KW - Immunoediting

KW - Immunosurveillance

KW - Mycosis fungoides

KW - Th1

U2 - 10.3390/cancers12082007

DO - 10.3390/cancers12082007

M3 - Review

C2 - 32707930

AN - SCOPUS:85088314441

VL - 12

SP - 1

EP - 21

JO - Cancers

JF - Cancers

SN - 2072-6694

IS - 8

M1 - 2007

ER -