Paediatric Mycosis Fungoides: Clinical Variants, Treatment Modalities and Response to Therapy
Keywords:mycosis fungoides, folliculotropic, paediatric, cutaneous lymphoma, phototherapy
Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological characteristics, as well as the treatment modalities and response to therapy of paediatric patients with mycosis fungoides. This retrospective cohort study reviewed the records of 37 paediatric patients treated at Rambam Medical Center, Israel, between 2013 and 2021. Extracted data included epidemiology, clinical presentation, histological reports, infiltrate clonality status, treatment modalities and response to therapy. The mean follow-up period was 60 months. All patients were diagnosed with stage IA or IB disease. Folliculotropic mycosis fungoides was the most prevalent variant (49%). Most patients were treated with phototherapy (90%), with a response rate of 85%, and a complete response rate of 55% after the first course. There were no significant differences in response to phototherapy between the folliculotropic or other variants (p = 0.072). Similarly, delayed diagnosis, atopic diathesis, clonality, phototherapy type or number of treatments, were not associated with response to therapy, while protracted phototherapy was associated with prolonged remission. In conclusion, mycosis fungoides in the paediatric population is an indolent disease with a favourable prognosis and potentially prolonged response to phototherapy.
Ceppi F, Pope E, Ngan B, Abla O. Primary cutaneous lymphomas in children and adolescents. Pediatr Blood Cancer 2016; 63: 1886-1894.
Assaf C, Gellrich S, Steinhoff M, Nashan D, Weisse F, Dippel E, et al. Cutaneous lymphomas in Germany: an analysis of the Central Cutaneous Lymphoma Registry of the German Society of Dermatology (DDG). J Dtsch Dermatol Ges 2007; 5: 662-668.
Jung JM, Lim DJ, Won CH, Chang SE, Lee MW, Lee WJ. Mycosis fungoides in children and adolescents: a systematic review. JAMA Dermatol 2021; 157: 431-438.
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.
Virmani P, Levin L, Myskowski PL, Flores E, Marchetti MA, Lucas AS, et al. clinical outcome and prognosis of young patients with mycosis fungoides. Pediatr Dermatol 2017; 34: 547-553.
Wu JH, Cohen BA, Sweren RJ. Mycosis fungoides in pediatric patients: clinical features, diagnostic challenges, and advances in therapeutic management. Pediatr Dermatol 2020; 37: 18-28.
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.
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127: 2375-2390.
Larocca C, Kupper T, Mycosis fungoides and Sézary syndrome: an update. Hematol Ocol Clin North Am 2019; 33: 103-120.
Nuckols JD, Shea CR, Horenstein MG, Burchette JL, Prieto VG. Quantitation of intraepidermal T-cell subsets in formalin-fixed, paraffin-embedded tissue helps in the diagnosis of mycosis fungoides, J Cutan Pathol 1999; 26: 169-175.
Boccara O, Blanche S, de Prost Y, Brousse N, Bodemer C, Fraitag S, Cutaneous hematologic disorders in children, Pediatr Blood Cancer 2012; 58: 226-232.
Boulos S, Vaid R, Aladily TN, Ivan DS, Talpur R, Duvic M. Clinical presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: a case series of 34 patients. J Am Acad Dermatol 2014; 71: 1117-1126.
Koh M J-A, Chong W-S. Narrow-band ultraviolet B phototherapy for mycosis fungoides in children. Clin Exp Dermatol 2014; 39: 474-478.
Brazzelli V, Bernacca C, Segal A, Barruscotti S, Bolcato V, Michelerio A, et al. Photo-photochemotherapy in juvenile-onset mycosis fungoides: a retrospective study on 9 patients. J Pediatr Hematol Oncol 2019; 41: 34-37.
Krutmann J. Therapeutic photoimmunology: photoimmunological mechanisms in photo(chemo)therapy. J Photochem Photobiol B 1998; 44: 159-164.
Nanda A, AlSaleh QA, Al-Ajmi H, Al-Sabah H, Elkashlan M, Al-Shemmari S, et al. Mycosis fungoides in Arab children and adolescents: a report of 36 patients from Kuwait, Pediatr Dermatol 2010; 27: 607-613.
Yazganoglu KD, Topkarci Z, Buyukbabani N, Baykal C. Childhood mycosis fungoides: a report of 20 cases from Turkey. J Eur Acad Dermatol Venereol 2013; 27: 295-300.
Ramsay DL, Parnes RE, Dubin N. Response of mycosis fungoides to topical chemotherapy with mechlorethamine, Arch Dermatol 1984; 120: 1585-1590.
Micaily B, Miyamoto C, Kantor G, Lessin S, Rook A, Brady L, et al. Radiotherapy for unilesional mycosis fungoides. Int J Radiat Oncol Biol Phys 1998; 42: 361-364.
Huen AO, Kim EJ. The role of systemic retinoids in the treatment of cutaneous T-cell lymphoma. Dermatol Clin 2015; 33: 715-729.
Zackheim HS, Kashani-Sabet M, McMillan A. Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients, J Am Acad Dermatol 2003; 49: 873-878.
Whittaker S, Hoppe R, HM. How I treat mycosis fungoides and Sézary syndrome. Blood 2016; 127: 3142-3153.
Ben-Amitai D, Michael D, Feinmesser M, Hodak E. Juvenile mycosis fungoides diagnosed before 18 years of age. Acta Derm Venereol 2003; 83: 451-456.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas, Blood 2005; 105: 3768-3785.
Hodak E, Amitay-Laish I. Mycosis fungoides: a great imitator. Clin Dermatol 2019; 37: 255-267.
Walia R, Yeung CCS. An update on molecular biology of cutaneous T cell lymphoma. Front Oncol 2019; 9: 1558.
Ponti R, Quaglino P, Novelli M, Fierro MT, Comessatti A, Peroni A, et al. T-cell receptor gamma gene rearrangement by multiplex polymerase chain reaction/heteroduplex analysis in patients with cutaneous T-cell lymphoma (mycosis fungoides/Sézary syndrome) and benign inflammatory disease: correlation with clinical, histological and immunophenotypical findings. Br J Dermatol 2005; 153: 565-573.
Travaglino A, Varricchio S, Pace M, Russo D, Picardi M, Baldo A. Borrelia burgdorferi in primary cutaneous lymphomas: a systematic review and meta-analysis. J Dtsch Dermatol Ges 2020; 18: 1379-1384.
Brunner PM, Guttman-Yassky E, Leung DYM. The immunology of atopic dermatitis and its reversibility with broad-spectrum and targeted therapies. J Allergy Clin Immunol 2017; 139: S65-S76.
Zhou H, Luo ZD, Tang XH, Han JD, Gao Q. Folliculotropic mycosis fungoides associated with atopic dermatitis. Australas J Dermatol 2018; 59: e143-e145.
Olsen EA, Hodak E, Anderson T, Carter JB, Henderson M, Cooper K, et al. Guidelines for phototherapy of mycosis fungoides and Sézary syndrome: a consensus statement of the United States Cutaneous Lymphoma Consortium. J Am Acad Dermatol 2016; 74: 27-58.
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