Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
Keywords:mycosis fungoides, hypopigmentation, cutaneous T-cell lymphomas, interferon
Hypopigmented mycosis fungoides is a rare form of mycosis fungoides that is characterized by achromic lesions, early onset of disease, a predilection for darker skinned populations, and a predominance of CD8+ T cells. Due to the rarity and heterogeneous presentation of hypopigmented mycosis fungoides, there are no criteria that clearly define the clinical characteristics and treatment regimens for this condition. This retrospective study of 44 paediatric patients with hypopigmented mycosis fungoides aimed to summarize their epidemiological and clinical characteristics and assess the effectiveness and safety of different treatment regimens. Clinical manifestations were further classified into 3 morphological groups: hypopigmented lesions, papules overlying hypopigmented lesions, and erythematous plaques overlying hypopigmented lesions. In addition, the results of this study suggest that interferon alpha might be an effective and well-tolerated therapy that could shorten the treatment time to complete response compared with other treatments. Maintenance therapy and long-term follow-up reduced the recurrence rate.
Dummer R, Vermeer MH, Scarisbrick JJ, Kim YH, Stonesifer C, Tensen CP, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers 2021 7: 61.
Martínez Villarreal A, Gantchev J, Lagacé F, Barolet A, Sasseville D, Ødum N, et al. Hypopigmented mycosis fungoides: loss of pigmentation reflects antitumor immune response in young patients. Cancers (Basel) 2020; 12.
Furlan FC, de Paula Pereira BA, da Silva LF, Sanches JA. Loss of melanocytes in hypopigmented mycosis fungoides: a study of 18 patients. J Cutan Pathol 2014; 41: 101-107.
Amorim GM, Niemeyer-Corbellini JP, Quintella DC, Cuzzi T, Ramos ESM. Hypopigmented mycosis fungoides: a 20-case retrospective series. Int J Dermatol 2018; 57: 306-312.
Castano E, Glick S, Wolgast L, Naeem R, Sunkara J, Elston D, et al. Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study. J Cutan Pathol 2013; 40: 924-934.
Stone ML, Styles AR, Cockerell CJ, Pandya AG. Hypopigmented mycosis fungoides: a report of 7 cases and review of the literature. Cutis 2001; 67: 133-138.
Ardigó M, Borroni G, Muscardin L, Kerl H, Cerroni L. Hypopigmented mycosis fungoides in Caucasian patients: a clinicopathologic study of 7 cases. J Am Acad Dermatol 2003; 49: 264-270.
Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019; 133: 1703-1714.
Rodney IJ, Kindred C, Angra K, Qutub ON, Villanueva AR, Halder RM. Hypopigmented mycosis fungoides: a retrospective clinicohistopathologic study. J Eur Acad Dermatol Venereol 2017; 31: 808-814.
Olsen EA, Whittaker S, Kim YH, Duvic M, Prince HM, Lessin SR, et al. Clinical end points and response criteria in mycosis fungoides and Sézary syndrome: a consensus statement of the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the European Organisation for Research and Treatment of Cancer. J Clin Oncol 2011; 29: 2598-2607.
Reiter O, Amitay-Laish I, Oren-Shabtai M, Feinmesser M, Ben-Amitai D, Hodak E. Paediatric mycosis fungoides - characteristics, management and outcomes with particular focus on the folliculotropic variant. J Eur Acad Dermatol Venereol 2022; 36: 671-679.
Hodak E, Amitay-Laish I, Feinmesser M, Davidovici B, David M, Zvulunov A, et al. Juvenile mycosis fungoides: cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol 2014; 70: 993-1001.
Shi HZ, Jiang YQ, Xu XL, Zhang W, Song H, Wang XP, et al. Hypopigmented mycosis fungoides: a clinicopathological review of 32 patients. Clin Cosmet Investig Dermatol 2022; 15: 1259-1264.
Domínguez-Gómez MA, Baldassarri-Ortego LF, Morales-Sánchez MA. Hypopigmented mycosis fungoides: a 48-case retrospective series. Australas J Dermatol 2021; 62: e419-e420.
Chen Y, Xu J, Qiu L, Fu L, Liang Y, Wei L, et al. Hypopigmented mycosis fungoides: a clinical and histopathology analysis in 9 children. Am J Dermatopathol 2021; 43: 259-265.
Furlan FC, Pereira BA, Sotto MN, Sanches JA. Hypopigmented mycosis fungoides versus mycosis fungoides with concomitant hypopigmented lesions: same disease or different variants of mycosis fungoides? Dermatology 2014; 229: 271-274.
Hassab-El-Naby HM, El-Khalawany MA. Hypopigmented mycosis fungoides in Egyptian patients. J Cutan Pathol 2013; 40: 397-404.
Wongpraparut C, Setabutra P. Phototherapy for hypopigmented mycosis fungoides in Asians. Photodermatol Photoimmunol Photomed 2012; 28: 181-186.
Kanokrungsee S, Rajatanavin N, Rutnin S, Vachiramon V. Efficacy of narrowband ultraviolet B twice weekly for hypopigmented mycosis fungoides in Asians. Clin Exp Dermatol 2012; 37: 149-152.
Khopkar U, Doshi BR, Dongre AM, Gujral S. A study of clinicopathologic profile of 15 cases of hypopigmented mycosis fungoides. Indian J Dermatol Venereol Leprol 2011; 77: 167-173.
Xie M, Huang X, Ye X, Qian W. Prognostic and clinicopathological significance of PD-1/PD-L1 expression in the tumor microenvironment and neoplastic cells for lymphoma. Int Immunopharmacol 2019; 77: 105999.
Di Raimondo C, Rubio-Gonzalez B, Palmer J, Weisenburger DD, Zain J, Wu X, et al. Expression of immune checkpoint molecules programmed death protein 1, programmed death-ligand 1 and inducible T-cell co-stimulator in mycosis fungoides and Sézary syndrome: association with disease stage and clinical outcome. Br J Dermatol 2022; 187: 234-243.
Nguyen GH, Olson LC, Magro CM. Upregulation of inhibitory signaling receptor programmed death marker-1 (PD-1) in disease evolution from cutaneous lymphoid dyscrasias to mycosis fungoides and Sezary's syndrome. Ann Diagn Pathol 2017; 28: 54-59.
Allen PB, McCook-Veal AA, Switchenko JM, Paulino DM, Niyogusaba T, Baird KM, et al. Staging lymph nodes and blood at diagnosis in mycosis fungoides identifies patients at increased risk of progression to advanced stage: a retrospective cohort study. Cancer 2023; 129: 541-550.
Zimmermann C, Boisson M, Ram-Wolff C, Sadoux A, Louveau B, Vignon-Pennamen MD, et al. Diagnostic performance of high-throughput sequencing of the T-cell receptor beta gene for the diagnosis of cutaneous T-cell lymphoma. Br J Dermatol 2021; 185: 679-680.
Marks E, Wang Y, Shi Y, Susa J, Jacobson M, Goldstein DY. Specific TCR gene rearrangements in mycosis fungoides: does advanced clinical stage show a preference? J Clin Pathol 2018; 71: 1072-1077.
Calvani J, de Masson A, de Margerie-Mellon C, de Kerviler É, Ram-Wolff C, Gruber A, et al. Image-guided lymph node core-needle biopsy predicts survival in mycosis fungoides and Sézary syndrome. Br J Dermatol 2021; 185: 419-427.
Jawed SI, Myskowski PL, Horwitz S, Moskowitz A, Querfeld C. 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; 70: 205.e201-216; quiz 221-202.
Dummer R, Vermeer MH, Scarisbrick JJ, Kim YH, Stonesifer C, Tensen CP, et al. Cutaneous T cell lymphoma. Nat Rev Dis Primers 2021; 7: 61.
Olsen EA, Whittaker S, Willemze R, Pinter-Brown L, Foss F, Geskin L, et al. Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC. Blood 2022; 140: 419-437.
Spaccarelli N, Rook AH. The use of interferons in the treatment of cutaneous T-cell lymphoma. Dermatol Clin 2015; 33: 731-745.
Olsen EA. Interferon in the treatment of cutaneous T-cell lymphoma. Dermatol Ther 2003; 16: 311-321.
Stadler R, Otte HG, Luger T, Henz BM, Kühl P, Zwingers T, et al. Prospective randomized multicenter clinical trial on the use of interferon-2a plus acitretin versus interferon -2a plus PUVA in patients with cutaneous T-cell lymphoma stages I and II. Blood 1998; 92: 3578-3581.
Furlan FC, Sanches JA. Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology. An Bras Dermatol 2013; 88: 954-960.
Akaraphanth R, Douglass MC, Lim HW. Hypopigmented mycosis fungoides: treatment and a 6(1/2)-year follow-up of 9 patients. J Am Acad Dermatol 2000; 42: 33-39.
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