The different faces of mycosis fungoides: results of a single-center study.
cutaneous lymphoma
mycosis fungoides; CTCL (lymphoma)
Journal
International journal of dermatology
ISSN: 1365-4632
Titre abrégé: Int J Dermatol
Pays: England
ID NLM: 0243704
Informations de publication
Date de publication:
Mar 2020
Mar 2020
Historique:
received:
31
01
2019
revised:
21
10
2019
accepted:
05
11
2019
pubmed:
30
11
2019
medline:
29
9
2020
entrez:
30
11
2019
Statut:
ppublish
Résumé
Mycosis fungoides (MF) accounts for the majority of cutaneous lymphomas. Apart from the predominant Alibert-Bazin type, several clinicopathological variants of diverse prevalence and biological behavior have been described. Data on clinical and epidemiological aspects of MF clinical subtypes are still weak. To outline the clinical and epidemiological profile of the different MF types in a large volume of Greek patients. Retrospective analysis of 688 MF cases treated in our lymphoma clinic. Epidemiological, clinical, pathological, and immunohistochemical data were retrieved. Six-hundred and thirty-six patients (416 males, 220 females) were included. The mean age at diagnosis was 60.2 years; the mean duration of disease prior to diagnosis was 63.2 months. Early-stage MF (I-IIA) involved 475 cases (74.7%). The prevalent type was classical MF (68.5%), followed by folliculotropic (17%), poikilodermic (5.5%), and psoriasiform (4.7%) MF. Atypical MF lesions as the sole manifestation of folliculotropic mycosis fungoides (FMF) - alopecia areata-like lesions (n = 10), keratosis pilaris-like lesions (n = 9) or acneiform rash (n = 4) - were also observed. Both poikilodermic and folliculotropic subtypes mainly involved younger patients. A significant diagnostic latency concerning poikilodermic and psoriasiform MF cases was recorded. Only 23 (3.3%) cases were of juvenile onset, with classical and poikilodermic MF equally affecting this age group, closely followed by FMF. Our study presents the whole clinical-epidemiological spectrum of MF in a large Greek cohort. The high prevalence of atypical MF manifestations characterized by early onset and indolent clinical course stood out among our FMF sample.
Sections du résumé
BACKGROUND
BACKGROUND
Mycosis fungoides (MF) accounts for the majority of cutaneous lymphomas. Apart from the predominant Alibert-Bazin type, several clinicopathological variants of diverse prevalence and biological behavior have been described. Data on clinical and epidemiological aspects of MF clinical subtypes are still weak.
AIM
OBJECTIVE
To outline the clinical and epidemiological profile of the different MF types in a large volume of Greek patients.
METHODS
METHODS
Retrospective analysis of 688 MF cases treated in our lymphoma clinic. Epidemiological, clinical, pathological, and immunohistochemical data were retrieved.
RESULTS
RESULTS
Six-hundred and thirty-six patients (416 males, 220 females) were included. The mean age at diagnosis was 60.2 years; the mean duration of disease prior to diagnosis was 63.2 months. Early-stage MF (I-IIA) involved 475 cases (74.7%). The prevalent type was classical MF (68.5%), followed by folliculotropic (17%), poikilodermic (5.5%), and psoriasiform (4.7%) MF. Atypical MF lesions as the sole manifestation of folliculotropic mycosis fungoides (FMF) - alopecia areata-like lesions (n = 10), keratosis pilaris-like lesions (n = 9) or acneiform rash (n = 4) - were also observed. Both poikilodermic and folliculotropic subtypes mainly involved younger patients. A significant diagnostic latency concerning poikilodermic and psoriasiform MF cases was recorded. Only 23 (3.3%) cases were of juvenile onset, with classical and poikilodermic MF equally affecting this age group, closely followed by FMF.
CONCLUSIONS
CONCLUSIONS
Our study presents the whole clinical-epidemiological spectrum of MF in a large Greek cohort. The high prevalence of atypical MF manifestations characterized by early onset and indolent clinical course stood out among our FMF sample.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
314-320Informations de copyright
© 2019 The International Society of Dermatology.
Références
Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768-3785.
Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms. Blood 2016; 127: 2375-2390.
Mitteldorf C, Grabbe S, Stadler R. [WHO classification and clinical spectrum of cutaneous lymphomas] Hautarzt 2017; 68: 682-695.
Cerroni L. Mycosis fungoides-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg 2018; 37: 2-10.
Kazakov DV, Burg G, Kempf W. Clinicopathological spectrum of mycosis fungoides. J Eur Acad Dermatol Venereol 2004; 18: 397-415.
Willemze R. Mycosis fungoides variants-clinicopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg 2018; 37: 11-17.
Martínez-Escala ME, González BR, Guitart J. Mycosis Fungoides Variants. Surg Pathol Clin 2014; 7: 169-189.
Ahn CS, ALSayyah A, Sangüeza OP. Mycosis fungoides: an updated review of clinicopathologic variants. Am J Dermatopathol 2014; 36: 933-948; quiz 949-951.
Zackheim HS, McCalmont TH. Mycosis fungoides: the great imitator. J Am Acad Dermatol 2002; 47: 914-918.
Nashan D, Faulhaber D, Ständer S, et al. Mycosis fungoides: a dermatological masquerader. Br J Dermatol 2007; 156: 1-10.
Olsen E, Vonderheid E, Pimpinelli N, et al. Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC). Blood 2007; 110: 1713-1722.
Nikolaou V, Papadavid E, Ekonomidi A, et al. Association of clinicopathological characteristics with secondary neoplastic lymphoproliferative disorders in patients with lymphomatoid papulosis. Leuk Lymphoma 2015; 56: 1303-1307.
Zackheim HS, Jones C, Leboit PE, et al. Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses for clonality. J Am Acad Dermatol 2003; 49: 620-623.
Baykal C, Atci T, Ozturk Sari S, et al. Underrecognized clinical features of folliculotropic mycosis fungoides: a large clinical series. J Dtsch Dermatol Ges 2017; 15: 289-299.
Muniesa C, Estrach T, Pujol RM, et al. Folliculotropic mycosis fungoides: clinicopathological features and outcome in a series of 20 cases. J Am Acad Dermatol 2010; 62: 418-426.
Scarisbrick JJ, Quaglino P, Prince HM, et al. The PROCLIPI international registry of early stage Mycosis Fungoides identifies substantial diagnostic delay in most patients. Br J Dermatol 2019; 181: 350-357.
Wieser I, Wang C, Alberti-Violetti S, et al. Clinical characteristics, risk factors and long-term outcome of 114 patients with folliculotropic mycosis fungoides. Arch Dermatol Res 2017; 309: 453-459.
Hodak E, Amitay-Laish I, Feinmesser M, et al. Juvenile mycosis fungoides: cutaneous T-cell lymphoma with frequent follicular involvement. J Am Acad Dermatol 2014; 70: 993-1001.
Mitteldorf C, Stadler R, Sander CA, et al. Folliculotropic mycosis fungoides. J Dtsch Dermatol Ges 2018; 16: 543-557.
Bagot M. Folliculotropic mycosis fungoides is a heterogenous group. Br J Dermatol 2017; 177: 17-18.
van Santen S, Roach RE, van Doorn R, et al. Clinical Staging and Prognostic Factors in Folliculotropic Mycosis Fungoides. JAMA Dermatol 2016; 152: 992-1000.
Nikolaou VA, Papadavid E, Katsambas A, et al. Clinical characteristics and course of CD8+ cytotoxic variant of mycosis fungoides: a case series of seven patients. Br J Dermatol 2009; 161: 826-830.
Abbott RA, Sahni D, Robson A, et al. Poikilodermatous mycosis fungoides: a study of its clinicopathological, immunophenotypic, and prognostic features. J Am Acad Dermatol 2011; 65: 313-319.
Donigan JM, Snowden C, Carter JB, et al. The temporal association between cutaneous T-cell lymphoma and psoriasis: implications for common biologic processes. J Eur Acad Dermatol Venereol 2016; 30: e31-e32.
Gelfand JM, Shin DB, Neimann AL, et al. The risk of lymphoma in patients with psoriasis. J Invest Dermatol 2006; 126: 2194-2201.
Nikolaou V, Marinos L, Moustou E, et al. Psoriasis in patients with mycosis fungoides: a clinicopathological study of 25 patients. J Eur Acad Dermatol Venereol 2017; 31: 1848-1852.
Papathemeli D, Georgiou E, Koletsa T, et al. Mycosis fungoides in patients with psoriasis: an ongoing issue. Eur J Dermatol 2018; 28: 235-236.
Martinez-Escala ME, Posligua AL, Wickless H, et al. Progression of undiagnosed cutaneous lymphoma after anti-tumor necrosis factor-alpha therapy. J Am Acad Dermatol 2018; 78: 1068-1076.
Lim HLJ, Tan EST, Tee SI, et al. Epidemiology and prognostic factors for mycosis fungoides and Sézary syndrome in a multi-ethnic Asian cohort: a 12-year review. J Eur Acad Dermatol Venereol. 2019; 33: 1513-1521.
Jang MS, Kang DY, Park JB, et al. Cutaneous T-cell lymphoma in Asians. ISRN Dermatol 2012; 2012: 575120.
Castano E, Glick S, Wolgast L, et al. Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study. J Cutan Pathol 2013; 40: 924-934.
Furlan FC, Sanches JA. Hypopigmented mycosis fungoides: a review of its clinical features and pathophysiology. An Bras Dermatol 2013; 88: 954-960.
Kempf W, Kazakov DV, Belousova IE, et al. Paediatric cutaneous lymphomas: a review and comparison with adult counterparts. J Eur Acad Dermatol Venereol 2015; 29: 1696-1709.
Gru AA, Dehner LP. Cutaneous hematolymphoid and histiocytic proliferations in children. Pediatr Dev Pathol 2018; 21: 208-251.