Epidemiology of cutaneous lymphoma

Vermeer, Maarten H.

doi:10.1111/bjd.19829

Cited by 8 publications

(8 citation statements)

References 7 publications

(23 reference statements)

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“…Regional differences have been reported, with a 15–17 % higher frequency in Asia and South America as compared to Europe [ 13 ]. The incidence of all cutaneous lymphomas has been reported to be about 0.64–0.87/100 000 per year for the USA [ 14 , 15 , 16 , 17 , 18 , 19 ], and 0.29–0.39/100 000 in Europe [ 20 , 21 , 22 , 23 ]. Current registry data from France, however, show an incidence of 0.96/100 000 for cutaneous lymphomas, which is near the incidence in the USA [ 24 ].…”

Section: Introduction and Epidemiologymentioning

confidence: 99%

Mycosis fungoides and Sézary syndrome

Jonak

Tittes

Brunner

et al. 2021

J Deutsche Derma Gesell

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Mycosis fungoides (MF) and Sézary syndrome (SS) are primary cutaneous T-cell lymphomas (CTCL) with not yet fully understood etiology and pathogenesis. Conceptually, MF and SS are classified as distinct entities arising from different T helper cell subsets. MF is the most common CTCL entity, while SS is very rare. MF presents clinically with patch, plaque and/or tumor stages, but can also evolve as erythroderma, which in turn is pathognomonic for SS. SS is characterized by a detectable tumor-cell burden (Sézary cells) in the peripheral blood consistent with advanced-stage disease and a poor prognosis. In early-stage disease of MF, which is the predominant form, the prognosis is generally favorable. However, in up to 30 % of patients, there is progression of skin lesions, which can ultimately lead to visceral involvement. The histological manifestation of MF can be subtle in early-stage disease and therefore a careful clinicopathological correlation is paramount. The treatment of MF/SS is dependent on the disease stage. Therapeutic options include both skin-directed and systemic regimens. Apart from allogeneic stem cell transplantation (alloSCT), there is as yet no curative therapy for MF/SS. Accordingly, the treatment approach is symptom oriented and aims to reduce the tumor burden and improve health-related quality of life. However, the therapeutic landscape for CTCL is constantly being expanded by the discovery of novel therapeutic targets.

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Section: Introduction and Epidemiologymentioning

confidence: 99%

Mycosis fungoides and Sézary syndrome

Jonak

Tittes

Brunner

et al. 2021

J Deutsche Derma Gesell

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“…In 2018 in France a PCL incidence of 0.96/100 000 was found. 3 A study on Dutch population reported an incidence of Mycosis fungoides (MF) in 0.19 and 0.46/100 000 persons in 2000 and 2020, respectively. 4 A recent analysis showed an incidence rate of CTCL of 1.02/100 000.…”

Section: Introductionmentioning

confidence: 99%

Primary cutaneous lymphoma patients seen at a referral dermatological centre in 1 year: A single‐centre observational retrospective cohort study of the diagnoses and staging, comorbidities and associated symptoms, treatment performed and clinical course

Macagno

Mastorino

Rubatto

et al. 2022

Acad Dermatol Venereol

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Importance Primary cutaneous lymphomas (PCL) are rare diseases, but the indolent course makes their prevalence high. Although there are many treatment options, no hierarchy is recommended. Objective To identify the burden of PCL and describe clinical‐pathologic features; associated comorbidities; analyse treatment approaches in real‐life and the parameters associated with the achievement of complete response (CR). Design, setting and participants In this study, all the PCL patients (384 patients) consecutively seen at the Dermatologic Clinic of the University of Turin from January 1, 2019 to December 31, 2019, with follow‐up updated to December 2020, were included. Main outcomes and measures Subtype of PCL, demographic data, time elapsed between first lesions and diagnosis, associated symptoms, comorbidities, staging at diagnosis, high‐grade transformation, blood involvement, stage progression, therapies used and response were assessed. Results 247 were cutaneous T‐cell lymphomas (CTCL, 64.3%), 137 cutaneous B‐cell lymphomas (CBCL, 35.7%) and the most frequent subtype was MF (48.4%). 62.3% of CTCL patients showed at least one comorbidity, mainly cardiovascular (28.7%), 20.2% show other not cutaneous neoplasms. The main approaches were skin‐directed therapies (topical steroids 65.6%; phototherapy 50.2%). 39.3% patients achieved a CR during the disease course. Pruritus, the presence of comorbidities and high‐grade transformation were factors associated with failure to achieve CR, whereas stage IA of MF was associated with greater achievement of CR. Conclusions and relevance The Th2 cytokine related development of pruritus could justify increased resistance to treatment, while the presence of associated comorbidities could reduce treatment options as well as treatment compliance.

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“…En otro estudio realizado también en los Estados Unidos, en 2011, se encontró que los LCCT representaron el 71% y los LCCB el 29% 10 . En concordancia con estos trabajos, en un estudio epidemiológico de LCP realizado en los Países Bajos en 2021 se encuentra una proporción mayor (75%) de LCCT que de LCCB (25%) 11 . Estos resultados contrastan con los de nuestro estudio, ya que si bien coincidimos en el predominio de los LCCT, en nuestra población pudimos observar una frecuencia relativa mayor de LCCT y menor de LCCB, correspondientes al 91.4% y el 8.6%, respectivamente (Tabla 4).…”

Section: Resultsunclassified