The First Year of the AEVD Primary Cutaneous Lymphoma Registry

Peñate, Yeray; Servitje, Octavio; Machan, Salma; Fernández-de-Misa, Ricardo; Estrach, M.T.; Acebo, Elvira; Mitxelena, J.; Ramón, M.; Flórez, Ángeles; Blanes, Mar; Morillo, Mercedes; Medina, Susana; Bassas, J.; Zayas, Ana; Espinosa, Patricia; Pérez, Amparo Sancho; Gónzalez-Romero, N.; Dominguez, J.D.; Muniesa, Cristina; Robles, J. Lopez; Combalía, Andrea; Yanguas, I.; Suh, H.; Polo‐Rodríguez, I.; Bielsa, Isabel; Mateu, A.; Ferrer, Berta; Descalzo, M.Á.; García‐Doval, I.; Ortiz‐Romero, Pablo L.

doi:10.1016/j.adengl.2018.06.003

Cited by 14 publications

(19 citation statements)

References 18 publications

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“…The number of included subjects ranged from 120 [ 11 ] to 4310 [ 10 ]. The study periods varied between four years and decades, and were not precise in two studies [ 17 , 18 ]. Eight reports were considered representative by the authors [ 10 , 19 , 20 , 21 , 22 , 23 , 24 , 25 ].…”

Section: Resultsmentioning

confidence: 99%

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients

Dobos

Pohrt

Ram‐Wolff

et al. 2020

Cancers

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Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of rare diseases. Many studies have reported on local epidemiology or geographic clustering, however we lack information from a global perspective. A systematic review and meta-analysis was conducted in Medline and the Cochrane Library based on a previously registered protocol and according to the preferred reporting of items for systematic reviews and meta-analyses (PRISMA). We selected publications that enrolled at least 100 patients with primary cutaneous lymphomas according to the current classifications. The relative frequencies (proportions) of subtypes were compared between studies and geographic regions in a meta-analysis. In total, 26 studies met our inclusion criteria, reporting on altogether 16,953 patients. Within primary cutaneous lymphomas, CTCL appeared to be 15% more frequent in Asian populations. Mycosis fungoides (MF) accounted for 62% of CTCL, with an important heterogeneity in frequencies between studies and continents. The proportion of Sézary syndrome (SS) was 3%, stable worldwide. Rare CTCL, such as NK/T-cell lymphoma or subcutaneous panniculitis-like lymphoma, were more frequent in Asian studies. This global meta-analysis of CTCL confirmed the predominance of CTCL among primary cutaneous lymphomas (83% on average) in the three analyzed continents, most of which were MF cases. It revealed the same proportions of SS across continents, and the heterogeneity of MF frequencies, suggesting the possible role of environmental factors in the pathophysiology of the latter. Registration number: CRD42020148295 (PROSPERO).

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Section: Resultsmentioning

confidence: 99%

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients

Dobos

Pohrt

Ram‐Wolff

et al. 2020

Cancers

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“…El grupo de LCP más frecuente en la serie de los autores fue el de los LCPCT con el 66.3% de los pacientes, y la MF fue el tipo más frecuente, con 58.2% del total de linfomas y el 70% de los LCPCT, de forma similar a lo publicado tanto en series internacionales 1 como en España 6 . Las ELPC CD30+ fueron los siguientes en frecuencia (18.2%) y la PL fue la más común.…”

Section: Discussionunclassified

“…Los LCPCB representaron el 33.7% de los linfomas, y el LCPZM fue el más frecuente (17.9% del total) hasta alcanzar el 74% de este grupo de linfomas. Esto contrasta con lo publicado en estudios anteriores en los que el LPCCF era el más común 1 , pero se halla en concordancia con lo observado en la serie del registro español 6 . En este artículo se explica la tendencia al equilibrio de los dos grupos de linfomas tras el predominio de los LCPCF por el hecho de la baja incidencia de factores etiológicos como la Borrelia burgdorferi en España y una mayor capacidad para diagnosticar procesos reactivos que antes se diagnosticaban como LCPCF.…”

Section: Discussionunclassified

“…La clasificación, controvertida y en constante cambio durante años, tiene en cuenta las características clínicas, histopatológicas, inmunofenotípicas y genéticas, con pronósticos y tratamientos específicos para cada linfoma [3][4][5][6][7][8][9] . Esta clasificación la han consensuado la Organización Mundial de la Salud (WHO) y la European Organisation for Research and Treatment of Cancer (EORTC), incluida además en la clasificación de los linfomas sistémicos (Tabla 1) 1 .…”

Section: Introductionunclassified

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Demora en el diagnóstico de los linfomas cutáneos primarios

Medina-Montalvo¹,

Polo-Rodrguez²,

Gmez-Zubiaur³

et al. 2021

MCUT

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Introducción: Los linfomas cutáneos son un grupo heterogéneo de linfomas no Hodgkin que se manifiestan en la piel, que es la localización extranodal más frecuente después del tracto gastrointestinal. Son linfomas cutáneos primarios (LCP) los que se hallan exclusivamente en la piel al momento del diagnóstico. Objetivo: Conocer el tiempo de demora diagnóstica de los LCP y su relación con el estadio al diagnóstico. Material y métodos: Estudio observacional descriptivo de corte transversal retrospectivo. Resultados: 83 pacientes diagnosticados con LCP, el 66.3% de linfomas cutáneos primarios de células T (LCPCT) y el 33.7% de linfomas cutáneos primarios de células B (LCPCB). El tiempo medio transcurrido desde la aparición de los primeros síntomas hasta el diagnóstico fue de 39.77 meses, con 42.93 meses para los LCPCT y 57 meses para los LCPCB. No se observó significación estadística entre un menor tiempo transcurrido hasta el diagnóstico y el estadio diagnóstico de la micosis fungoide (MF). No se observó una relación estadística entre el tiempo hasta el diagnóstico y el estadio de los casos de LCP centrofolicular (LCPCF) y LCP de la zona marginal (LCPZM). Conclusiones: La demora diagnóstica es >4 años para la MF y ~3 años para los LCPZM/LCPCF. A pesar de ello, en este grupo de linfomas indolentes, el diagnóstico se establece a menudo en estadios tempranos.

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“…Primary cutaneous CD30+ lymphoproliferative disorders are the second most common form of cutaneous T‐cell lymphoma, accounting for 11% to 20% of all primary cutaneous lymphomas. Lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (PCALCL) are the main subtypes, but also borderline lesions exist .…”

Section: Introductionmentioning

confidence: 99%

Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma

Fernández-de-Misa

Hernández‐Machín²,

Combalía

et al. 2019

Acad Dermatol Venereol

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Background Reliable prognostic factors for patients with primary cutaneous anaplastic large cell lymphoma (PCALCL) are lacking. Objective To identify prognostic factors for specific survival in patients with PCALCL. Methods Using the convenience sampling method, patients with PCALCL diagnosed from May 1986 to August 2017 in 16 University Departments were retrospectively reviewed. Results One hundred eight patients were included (57 males). Median age at diagnosis was 58 years. All of them showed T1‐3N0M0 stages. Seventy per cent of the cases presented with a solitary lesion, mostly at the limbs. Complete response rate after first‐line treatment was 87%, and no advantage was observed for any of them (surgery, radiotherapy, chemotherapy or other approaches). Nodal and visceral progression rate was 11% and 2%, respectively. 5‐year specific survival (SSV) reached 93%; 97% for T1 patients and 84% for T2/T3 patients (P = 0.031). Five‐year SSV for patients developing early cutaneous relapse was 64%; for those with late or no relapse, 96% (P = 0.001). Estimated median SSV for patients showing nodal progression was 103 months (95% CI: 51–155 months); for patients without nodal progression, estimated SSV did not reach the median (P < 0.001). Nodal progression was an independent predictive parameter for shorter survival (P = 0.011). Conclusion Multiple cutaneous lesions at presentation, early skin relapse and nodal progression portrait worse prognosis in patients with PCALCL.

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The First Year of the AEVD Primary Cutaneous Lymphoma Registry

Cited by 14 publications

References 18 publications

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients

Epidemiology of Cutaneous T-Cell Lymphomas: A Systematic Review and Meta-Analysis of 16,953 Patients

Demora en el diagnóstico de los linfomas cutáneos primarios

Prognostic factors in patients with primary cutaneous anaplastic large cell lymphoma: a multicentric, retrospective analysis of the Spanish Group of Cutaneous Lymphoma

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