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Los linfomas cutáneos primarios (LCP) son un conjunto de entidades poco frecuentes. En febrero del 2018 se describieron los resultados del primer año de funcionamiento del Registro de ...linfomas cutáneos primarios de la AEDV. En el presente trabajo actualizamos los resultados tras 5 años de funcionamiento.
Registro de enfermedad de pacientes con LCP. Se recogieron datos prospectivamente de los pacientes, incluyendo diagnóstico, tratamientos, pruebas realizadas y estado actual del paciente. Se realizó un análisis descriptivo.
En diciembre del 2021 se había incluido a un total de 2020 pacientes en el Registro, pertenecientes a 33 hospitales españoles. El 59% fueron hombres, la edad media fue de 62,2 años. Se agruparon en 4grandes grupos diagnósticos: micosis fungoide/síndrome de Sézary (1.112, 55%), LCP de células B (547, 27,1%), trastornos linfoproliferativos de células T CD30+(222, 11%) y otros linfomas T (116, 5,8%). La mayoría presentó estadio T1, encontrándose actualmente casi el 75% en remisión completa (43,5%) o enfermedad estable (EE; 27%). Los tratamientos más usados fueron corticoides tópicos (1.369, 67,8%), fototerapia (890, 44,1%), cirugía (412, 20,4%) y radioterapia (384, 19%).
Las características del paciente con LCP en España no difieren de otras series. El mayor tamaño del registro permite precisar mejor los datos con respecto a los resultados del primer año. Este registro facilita al grupo de linfomas de la AEDV realizar investigación clínica, surgiendo ya trabajos publicados de dicho registro.
Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years.
RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years.
Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 67.8%), phototherapy (890 patients 44.1%), surgery (412 patients 20.4%), and radiotherapy (384 patients 19%).
The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.
We describe the case of a 17-year-old patient with rapidly progressing and aggressive mycosis fungoides, with multiple cutaneous tumors and large cell transformation. She was initially treated with 3 ...cycles of high-dose chemotherapy with mega-CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) without response, leading to the decision to undertake autologous hematopoietic stem cell transplantation. Partial remission of the disease was achieved with this treatment and subsequent introduction of oral bexarotene led to complete remission, which has been maintained for more than 3 years with good tolerance of oral therapy. We discuss the advantages and disadvantages of autologous hematopoietic stem cell transplantation and the use of oral bexarotene.
Exponemos el caso de una paciente de 17 años que presentó una micosis fungoide de evolución rápida y agresiva, con múltiples tumores cutáneos y transformación fenotípica celular a linfoma de células grandes. Fue tratada inicialmente con 3 ciclos de quimioterapia en dosis altas (MEGA-CHOP) sin respuesta, por lo que se decidió realizar un trasplante autólogo de progenitores hematopoyéticos. Este tratamiento consiguió una remisión parcial de la enfermedad, y con la posterior introducción del bexaroteno oral se logró la remisión completa, que se ha mantenido durante más de 3 años con muy buena tolerancia al bexaroteno oral.
Analizamos las ventajas y desventajas de la utilización del trasplante autólogo de progenitores hematopoyéticos y del uso de bexaroteno oral.
Atopic dermatitis (AD) is one of the most prevalent skin diseases, but there are numerous knowledge gaps surrounding the impact this disease has on quality of life (QoL), mental health, and ...out-of-pocket expenses involved in the management of AD. The available scientific evidence on the multidimensional burden of AD is usually based on studies with measures reported by patients themselves.
In this context, the MEASURE-AD trial was developed as a cross-sectional, multicenter, multinational trial using patient- and physician-reported measures to characterize the multidimensional burden of AD in adults with moderate-to-severe AD.
This paper presents the results of the Spanish cohort. We found that Spanish adults with moderate-to-severe AD and high EASI score (21.1-72) had a significantly increased disease burden, high severity of symptoms such as itch and sleep disturbances, impaired mental health and QoL, higher use of health care resources, and more out-of-pocket expenses than patients with low EASI scores (0-7 or 7.1-21).
This study provides information to better understand disease burden, and identify aspects to be improved in the management of AD.
El bexaroteno está aprobado para el tratamiento de linfomas cutáneos de células T (LCCT) en estadio avanzado desde 1999. Sin embargo, se han publicado muy pocos datos sobre su seguridad y eficacia en ...el largo plazo. El objetivo del siguiente trabajo es determinar la eficacia y seguridad del bexaroteno en la serie de casos del Grupo Español de Linfomas Cutáneos (GELC) en su uso a largo plazo para el tratamiento de CTCL.
Realizamos una revisión retrospectiva multicéntrica de 216 pacientes con micosis fungoide (174) o síndrome de Sézary (42) tratados con bexaroteno solo o en combinación con otras terapias en 19 hospitales universitarios de referencia en España durante 10 años.
Se incluyeron 133 hombres (62%) y 83 mujeres (38%), con una edad media de 63,5 años (27-95). El 45% recibió monoterapia con bexaroteno durante todo el período de estudio, el 22% comenzó con bexaroteno pero posteriormente recibió una terapia adicional, el 13% estaba bajo otro tratamiento pero en algún momento se añadió bexaroteno y el 20% restante recibió una terapia combinada desde el principio. La mediana de duración del tratamiento fue de 20,78 meses (1-114). La tasa de respuesta global fue del 70,3%. Se lograron respuestas completas y parciales en el 26% y el 45% de los pacientes, respectivamente. El tratamiento fue bien tolerado; las toxicidades más frecuentes fueron la hipertrigliceridemia (79%), la hipercolesterolemia (71%) y el hipotiroidismo (52%). No se observaron efectos secundarios de grado 5 relacionados con el tratamiento.
Nuestro estudio confirma que el bexaroteno es una terapia segura y eficaz para el tratamiento a largo plazo de CTCL.
Bexarotene has been approved to treat advanced stage cutaneous T-cell lymphomas (CTCL) since 1999. However, very few data have been published on its long-term safety and efficacy profile. The aim of this study is to determine the tolerability to bexarotene and outcomes by collecting the 2nd largest case series to date on its long-term use vs CTCL.
This was a multicenter retrospective review of 216 patients with mycosis fungoides (174), or Sézary syndrome (42) on a 10-year course of bexarotene alone or in combination with other therapies at 19 tertiary referral teaching hospitals.
A total of 133 men (62%) and 83 women (38%) were included, with a mean age of 63.5 year (27–95). A total of 45% were on bexarotene monotherapy for the entire study period, 22% started on bexarotene but eventually received an additional therapy, 13% were on another treatment but eventually received bexarotene while the remaining 20% received a combination therapy since the beginning. The median course of treatment was 20.78 months (1–114); and the overall response rate, 70.3%. Complete and partial response rates were achieved in 26% and 45% of the patients, respectively. Treatment was well tolerated, being the most common toxicities hypertriglyceridemia (79%), hypercholesterolemia (71%), and hypothyroidism (52%). No treatment-related grade 5 adverse events were reported.
Our study confirms bexarotene is a safe and effective therapy for the long-term treatment of CTCL.
INTRODUCTION AND OBJECTIVESA great amount of information on systemic and biologic therapies for moderate to severe psoriasis is now available. However, applying the evidence in numerous clinical ...scenarios has engendered debate; under these circumstances, the consensus of experts is useful.MATERIAL AND METHODSA scientific committee systematically reviewed the literature relevant to 5 clinical scenarios. An online Delphi survey of dermatologists with experience treating moderate to severe psoriasis was then carried out in order to shed light on questions that remained unresolved by the available evidence.RESULTSTwenty-three dermatologists responded to the survey and consensus was reached on 37 (56%) of the 66 statements proposed. These results led to consensus on various clinical situations even though firm evidence was lacking. Thus, intermittent therapeutic regimens and strategies for reducing the intensity of treatment are considered appropriate for optimizing biologic treatment and reducing costs. The measurement of drug and antidrug antibody levels should be included routinely when following patients on biologics to treat psoriasis. Concomitant psoriatic arthritis or a history of cardiovascular conditions will influence the choice of biologic; in these situations, an agent with anti-tumor necrosis factor properties will be preferred. Tailored management is important when the patient is pregnant or intends to conceive; drug half-life and disease severity are important factors to take into consideration in these scenarios.CONCLUSIONSA combination of systematic review of the literature and structured discussion of expert opinion facilitates decision-making in specific clinical scenarios.
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En España, aunque el Ministerio de Sanidad elabora el informe de posicionamiento terapéutico (IPT) y las condiciones de reembolso de los fármacos, las Comunidades Autónomas (CC. AA.) ...gestionan los servicios de salud y deciden sobre las condiciones de prescripción en su ámbito territorial. El objetivo del estudio EQUIDAD fue describir los condicionantes para la prescripción de los nuevos fármacos en Dermatología en las CC. AA. y sus posibles diferencias.
Estudio transversal realizado en abril-mayo del 2023. Dos dermatólogos con responsabilidades directivas de cada Comunidad Autónoma (C. A.) informaron sobre los condicionantes autonómicos y locales en la prescripción de los fármacos cuyo IPT para el tratamiento de enfermedades dermatológicas fue publicado en los años 2016-2022. Los datos fueron recogidos mediante un cuestionario online.
Un total de 33 investigadores de 17 CC. AA. participaron en el estudio. Se observaron inequidades entre CC. AA. para el acceso a los nuevos fármacos. Existieron condicionantes autonómicos adicionales al IPT en psoriasis en el 64,7% de las CC. AA., siendo este porcentaje menor en dermatitis atópica (35,3%) o melanoma (11,8%). El más frecuente fue el requisito de un orden de prescripción previo para el uso del fármaco. En algunas CC. AA. se detectaron además variaciones y condicionantes locales (diferencias entre centros de una misma C. A.).
Existe una multiplicidad de criterios tanto a nivel autonómico como local que añade restricciones adicionales a las establecidas por los IPT y que plantean una situación de inequidad entre los pacientes y los profesionales de las diferentes CC. AA. en el acceso a los nuevos fármacos.
Although the Spanish Ministry of Health prepares national therapeutic positioning reports (TPRs) and drug reimbursement policies, each of the country's 17 autonomous communities (ACs) is responsible for health care services and prescription requirements in its territory. The aim of the EQUIDAD study was to describe and explore potential differences in prescription requirements for new dermatology drugs across the autonomous communities.
Cross-sectional study conducted in April and May, 2023. Two dermatologists with management responsibilities from each autonomous community reported on territorial and more local prescription requirements for drugs covered by national TPRs issued between 2016 and 2022.
Thirty-three researchers from 17 autonomous communities participated. The data submitted revealed between-community inequities in access to new drugs. Overall, 64.7% of the regions imposed additional prescription requirements to those mentioned in the TPRs for psoriasis. This percentage was lower for atopic dermatitis (35.3%) and melanoma (11.8%). The most common requirement for accessing a new drug was a previous prescription for another drug. Differences and additional requirements were also detected at
the local level (i.e., differences between hospitals within the same autonomous community).
Spain's autonomous communities have multiple regional and local prescription requirements that are not aligned with national TPR recommendations. These differences result in inequitable access to new drugs for both patients and practitioners across Spain.
Evaluation of disease severity is considered essential in the optimal management of psoriasis.
To describe the clinical characteristics and therapeutic profile of patients with moderate to severe ...psoriasis in Spain and to assess the impact of the disease on the patients' quality of life.
This was an observational, cross-sectional study carried out in 90 dermatology units in Spain in 2009. We included 442 patients diagnosed with moderate to severe psoriasis who had started treatment with systemic agents, phototherapy, and/or topical treatments between 2004 and 2006.
More severe psoriasis was significantly associated with the following: longer disease duration; higher prevalence of concomitant disease; greater involvement of the nails, scalp, flexures, palms, and soles; and poorer quality of life. In the 5 years before the start of the study, 68% of the patients had received conventional systemic treatments, 39.1% biologic agents, and 22.3% phototherapy. At present, 57.5% of the patients are being treated with biologic agents, 32.6% with conventional systemic treatments, and 11% with phototherapy.
Severity of psoriasis was associated with a marked impact on quality of life. Regardless of disease severity, psychiatric comorbidity was the strongest predictor of poor quality of life. On average, patients had received other treatments, such as conventional systemic treatments or phototherapy, for more than 2 years before switching to biologic agents for the first time.
To identify the most common therapeutic options for the treatment of early-stage mycosis fungoides in Spain, quantify their associated healthcare resource use and costs.
After reviewing the ...literature, a panel of 6 Spanish clinical dermatologists validated the treatments and healthcare resource use through a structured questionnaire. Individual responses were collected, analyzed and presented into a face-to-face meeting in order to reach a consensus. Cost categories considered were: drug acquisition and administration, photo/radiotherapy session and maintenance, clinical follow-up visits and laboratory tests. Costs were expressed in euros from 2018. The Spanish National Health System perspective was considered, taking into account direct health costs and time horizons of 1, 3 and 6 months.
Costs for the skin-directed treatments (SDT) assessed at 1, 3 and 6 months, were: Topical carmustine €6,593.36, €19,780.09 and €27,592.78; Phototherapy with psoralens and ultraviolet A light (PUVA) €1,098.68, €2,999.99 and €3,187.60; Narrow-band ultraviolet B phototherapy €1,657.47, €4,842.10 and €4,842.10; Total skin electron beam therapy (TSEBT) €6,796.45, €7,913.34 and €7,913.34. Cost for topical corticosteroids, being considered an adjuvant option, were €17.16, €51.49 and €102.97. Costs for the assessed systemic treatments alone or in combination with SDT at 1, 3 and 6 months, were: Systemic retinoids €2,026.03, €5,206.63 and €7,426.42; Systemic retinoids + PUVA phototherapy €3,066.50, €8,271.26 and €10,046.58; Interferon alfa + PUVA phototherapy €1,541.09, €5,167.57 and €6,404.55.
According to the Spanish clinical practice, phototherapies in monotherapy were the treatments with the lowest associated costs regardless of the time horizon considered. TSEBT turned out as the treatment with the highest associated costs when considering 1 month. However, while considering 3 and 6 months the treatment with the highest associated costs was topical carmustine. The results of this analysis may provide critical information to measure the disease burden, to detect unmet medical needs and to advocate towards better treatments for this rare disease.
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![[Artículo traducido] Experi...](http://api.summon.serialssolutions.com/2.0.0/image/issn/XR4PS5YY4K/0001-7310_1578-2190_1695-4734/small "[Artículo traducido] Experiencia con bexaroteno en linfoma cutáneo de células T: un estudio del Grupo Español de Linfomas Cutáneos (GELC)")
