Extracorporeal Photopheresis in Pediatric Graft-vs-Host Disease.

BACKGROUND: Extracorporeal photopheresis (ECP) is an immunomodulatory therapy used to treat graft-vs-host disease (GVHD) in adults and children. Few studies have examined its use in children. OBJECTIVE: To describe demographic characteristics, clinical response, adverse effects, and outcomes in a series of pediatric patients with acute or chronic GVHD treated with ECP. MATERIAL AND METHODS: We included all pediatric patients with acute or chronic GVHD treated with ECP by the dermatology department of Hospital Italiano de Buenos Aires between January 2012 and December 2018. We used the UVAR-XTS™ system (2 patients) and the CELLEX system (7 patients). Patients with acute GVHD received 2 sessions a week and were reassessed at 1 month, while those with chronic GVHD received 2 sessions every 2 weeks and were reassessed at 3 months. Treatment duration in both scenarios varied according to response. RESULTS: We evaluated 9 pediatric patients with corticosteroid-refractory, -dependent, and/or -resistant GVHD treated with ECP. Seven responded to treatment and 2 did not. Response was complete in 1 of the 9 patients with skin involvement and partial in 7. Complete response rates for the other sites of involvement were 60% (3/5) for the liver, 50% (1/2) for the gastrointestinal system, and 80% (4/5) for mucous membranes. Two patients died during the study period. CONCLUSION: ECP is a good treatment option for pediatric patients with acute or chronic GVHD.

Extracorporeal Photopheresis in Pediatric Graft-vs-Host Disease. / Fotoféresis extracorpórea en enfermedad injerto contra huésped en una población pediátrica.

BACKGROUND: Extracorporeal photopheresis (ECP) is an immunomodulatory therapy used to treat graft-vs-host disease (GVHD) in adults and children. Few studies have examined its use in children. OBJECTIVE: To describe demographic characteristics, clinical response, adverse effects, and outcomes in a series of pediatric patients with acute or chronic GVHD treated with ECP. MATERIAL AND METHODS: We included all pediatric patients with acute or chronic GVHD treated with ECP by the Dermatology Department of Hospital Italiano de Buenos Aires between January 2012 and December 2018. We used the UVAR-XTS™ system (2 patients) and the CELLEX system (7 patients). Patients with acute GVHD received 2 sessions a week and were reassessed at 1 month, while those with chronic GVHD received 2 sessions every 2 weeks and were reassessed at 3 months. Treatment duration in both scenarios varied according to response. RESULTS: We evaluated 9 pediatric patients with corticosteroid-refractory, -dependent, and/or -resistant GVHD treated with ECP. Seven responded to treatment and 2 did not. Response was complete in 1 of the 9 patients with skin involvement and partial in 7. Complete response rates for the other sites of involvement were 60% (3/5) for the liver, 50% (1/2) for the gastrointestinal system, and 80% (4/5) for mucous membranes. Two patients died during the study period. CONCLUSION: ECP is a good treatment option for pediatric patients with acute or chronic GVHD.

Treatment of early-stage mycosis fungoides: results from the PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study.

BACKGROUND: The PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study is a prospective analysis of an international database. Here we examine front-line treatments and quality of life (QoL) in patients with newly diagnosed mycosis fungoides (MF). OBJECTIVES: To identify (i) differences in first-line approaches according to tumour-nodes-metastasis-blood (TNMB) staging; (ii) parameters related to a first-line systemic approach and (iii) response rates and QoL measures. METHODS: In total, 395 newly diagnosed patients with early-stage MF (stage IA-IIA) were recruited from 41 centres in 17 countries between 1 January 2015 and 31 December 2018 following central clinicopathological review. RESULTS: The most common first-line therapy was skin-directed therapy (SDT) (322 cases, 81·5%), while a smaller percentage (44 cases, 11·1%) received systemic therapy. Expectant observation was used in 7·3%. In univariate analysis, the use of systemic therapy was significantly associated with higher clinical stage (IA, 6%; IB, 14%; IIA, 20%; IA-IB vs. IIA, P < 0·001), presence of plaques (T1a/T2a, 5%; T1b/T2b, 17%; P < 0·001), higher modified Severity Weighted Assessment Tool (> 10, 15%; ≤ 10, 7%; P = 0·01) and folliculotropic MF (FMF) (24% vs. 12%, P = 0·001). Multivariate analysis demonstrated significant associations with the presence of plaques (T1b/T2b vs. T1a/T2a, odds ratio 3·07) and FMF (odds ratio 2·83). The overall response rate (ORR) to first-line SDT was 73%, while the ORR to first-line systemic treatments was lower (57%) (P = 0·027). Health-related QoL improved significantly both in patients with responsive disease and in those with stable disease. CONCLUSIONS: Disease characteristics such as presence of plaques and FMF influence physician treatment choices, and SDT was superior to systemic therapy even in patients with such disease characteristics. Consequently, future treatment guidelines for early-stage MF need to address these issues.

Nodulo de sister Joseph como presentación del carcinoma de cuello uterino / Sister Joseph's nodule how presentation of the uterine cervix carcinoma

Las metástasis umbilicares son infrecuentes y reciben el nombre de nódulo de Sistes Joseph. Presentamos una mujer de 51 años que consultó por autodetectarse dos formaciones tumorales subcutáneas, la primera de tres meses de evolución en el ombligo y la restante en la pared anterior del tórax. La biopsis de la lesión umbilical imformó carcinoma epidermoide queratinizante subcutáneo diagnosticándose la neoplasia primaria en el cuello uterino. En el presente trabajo se realiza una reviosión bibliográfica del nódulo de Sister Joseph. Destacamos su escasa frecuencia como manifestación inicial del carcinoma de cuello uterino debiéndose considerar en el diagnóstico diferencial de los tumores de ombligo

Nodulo de sister Joseph como presentación del carcinoma de cuello uterino / Sister Josephs nodule how presentation of the uterine cervix carcinoma

Las metástasis umbilicares son infrecuentes y reciben el nombre de nódulo de Sistes Joseph. Presentamos una mujer de 51 años que consultó por autodetectarse dos formaciones tumorales subcutáneas, la primera de tres meses de evolución en el ombligo y la restante en la pared anterior del tórax. La biopsis de la lesión umbilical imformó carcinoma epidermoide queratinizante subcutáneo diagnosticándose la neoplasia primaria en el cuello uterino. En el presente trabajo se realiza una reviosión bibliográfica del nódulo de Sister Joseph. Destacamos su escasa frecuencia como manifestación inicial del carcinoma de cuello uterino debiéndose considerar en el diagnóstico diferencial de los tumores de ombligo(AU)

Vasculitis livedoide tratada con pentoxifilina / Livedo vasculitis: therapy with pentoxifylline

Comunicamos un caso de vasculitis livedoide (VL) de diez años de evolución en una mujer de 44 años sin colagenopatías u otras enfermedades sistémicas asociadas y que presentaba nula respuesta a los tratamientos previos (fundamentalmente aspirina-dipiridamol). La respuesta terapéutica a la pentoxifilina (ptx) fue excelente, con cicatrización total de las úlceras y un control evolutivo de 18 meses sin recaídas

Vasculitis livedoide tratada con pentoxifilina / Livedo vasculitis: therapy with pentoxifylline

Comunicamos un caso de vasculitis livedoide (VL) de diez años de evolución en una mujer de 44 años sin colagenopatías u otras enfermedades sistémicas asociadas y que presentaba nula respuesta a los tratamientos previos (fundamentalmente aspirina-dipiridamol). La respuesta terapéutica a la pentoxifilina (ptx) fue excelente, con cicatrización total de las úlceras y un control evolutivo de 18 meses sin recaídas (AU)