المؤلفون: Pascual Izquierdo, Cristina, Mingot Castellano, María Eva, Kerguelen Fuentes, Ana E., García-Arroba Peinado, José, Cid, Joan, Moraima Jimenez, Maria, Valcarcel, David, Gómez Seguí, Inés, de la Rubia, Javier, Martin, Paz, Goterris, Rosa, Hernández, Luis, Tallón, Inmaculada, Varea, Sara, Fernández, Marta, García Muñoz, Nadia, Vara, Míriam, Fernández Zarzoso, Miguel, García Candel, Faustino, Paciello, María Liz, García García, Irene, Zalba, Saioa, Campuzano, Verónica, Gala, José María, Vidán Estévez, Julia, Moreno Jiménez, Gemma, López Lorenzo, José Luis, González Arias, Elena, Freiría, Carmen, Solé, María, Ávila Idrovo, Laura Francisca, Hernández Castellet, José Carlos, Cruz, Naylen, Lavilla, Esperanza, Pérez Montaña, Albert, Atucha, Jon Ander, Moreno Beltrán, María Esperanza, Moreno Macías, Juán Ramón, Salinas, Ramón, del Rio Garma, Julio

المصدر: Blood Advances. Vol. 6, nº 24, December 2022, pp. 6219 - 6227

مصطلحات موضوعية: caplacizumab, prednisone, rituximab

الوصف: Immune thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy caused by anti-ADAMTS13 antibodies. Caplacizumab is approved for adults with an acute episode of iTTP in conjunction with plasma exchange (PEX) and immunosuppression. The objective of this study was to analyze and compare the safety and efficacy of caplacizumab vs the standard of care and assess the effect of the concomitant use of rituximab. A retrospective study from the Spanish TTP Registry of patients treated with caplacizumab vs those who did not receive it was conducted. A total of 155 patients with iTTP (77 caplacizumab, 78 no caplacizumab) were included. Patients initially treated with caplacizumab had fewer exacerbations (4.5% vs 20.5%; P < .05) and less refractoriness (4.5% vs 14.1%; P < .05) than those who were not treated. Time to clinical response was shorter when caplacizumab was used as initial treatment vs caplacizumab used after refractoriness or exacerbation. The multivariate analysis showed that its use in the first 3 days after PEX was associated with a lower number of PEX (odds ratio, 7.5; CI, 2.3-12.7; P < .05) and days of hospitalization (odds ratio, 11.2; CI, 5.6-16.9; P < .001) compared with standard therapy. There was no difference in time to clinical remission in patients treated with caplacizumab compared with the use of rituximab. No severe adverse event was described in the caplacizumab group. In summary, caplacizumab reduced exacerbations and refractoriness compared with standard of care regimens. When administered within the first 3 days after PEX, it also provided a faster clinical response, reducing hospitalization time and the need for PEX. ; 9 páginas

وصف الملف: application/pdf

العلاقة: http://hdl.handle.net/10498/29789Test

الإتاحة: https://doi.org/10.1182/bloodadvances.2022008028Test
http://hdl.handle.net/10498/29789Test

المؤلفون: Pascual Izquierdo, Cristina, Mingot Castellano, María Eva, Kerguelen Fuentes, Ana E., García Arroba Peinado, José, Cid Vidal, Joan, Jimenez, Maria Moraima, Valcarcel, David, Gómez Seguí, Inés, Rubia, Javier de la, Martin, Paz, Goterris, Rosa, Hernández, Luis, Tallón, Inmaculada, Varea, Sara, Fernández, Marta, García Muñoz, Nadia, Vara, Míriam, Fernández Zarzoso, Miguel, García Candel, Faustino, Paciello, María Liz, García García, Irene, Zalba, Saioa, Campuzano, Verónica, Gala, José María, Vidán Estévez, Julia, Moreno Jiménez, Gemma, López Lorenzo, José Luis, González Arias, Elena, Freiría, Carmen, Solé, María, Ávila Idrovo, Laura Francisca, Hernández Castellet, José Carlos, Cruz, Naylen, Lavilla, Esperanza, Pérez Montaña, Albert, Atucha, Jon Ander, Moreno Beltrán, María Esperanza, Moreno Macías, Juán Ramón, Salinas, Ramón, Rio Garma, Julio del, Spanish Apheresis Group (GEA), Spanish Thrombotic Thrombocytopenic Purpura Registry (REPTT)

المصدر: Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

مصطلحات موضوعية: Rituximab, Trombosi, Assaigs clínics, Thrombosis, Clinical trials

الوصف: Immune thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy caused by anti-ADAMTS13 antibodies. Caplacizumab is approved for adults with an acute episode of iTTP in conjunction with plasma exchange (PEX) and immunosuppression. The objective of this study was to analyze and compare the safety and efficacy of caplacizumab vs the standard of care and assess the effect of the concomitant use of rituximab. A retrospective study from the Spanish TTP Registry of patients treated with caplacizumab vs those who did not receive it was conducted. A total of 155 patients with iTTP (77 caplacizumab, 78 no caplacizumab) were included. Patients initially treated with caplacizumab had fewer exacerbations (4.5% vs 20.5%; P < .05) and less refractoriness (4.5% vs 14.1%; P < .05) than those who were not treated. Time to clinical response was shorter when caplacizumab was used as initial treatment vs caplacizumab used after refractoriness or exacerbation. The multivariate analysis showed that its use in the first 3 days after PEX was associated with a lower number of PEX (odds ratio, 7.5; CI, 2.3-12.7; P < .05) and days of hospitalization (odds ratio, 11.2; CI, 5.6-16.9; P < .001) compared with standard therapy. There was no difference in time to clinical remission in patients treated with caplacizumab compared with the use of rituximab. No severe adverse event was described in the caplacizumab group. In summary, caplacizumab reduced exacerbations and refractoriness compared with standard of care regimens. When administered within the first 3 days after PEX, it also provided a faster clinical response, reducing hospitalization time and the need for PEX.

وصف الملف: 9 p.; application/pdf

العلاقة: Reproducció del document publicat a: https://doi.org/10.1182/bloodadvances.2022008028Test; Blood Advances, 2022, vol. 6, num. 24, p. 6219-6227; https://doi.org/10.1182/bloodadvances.2022008028Test; http://hdl.handle.net/2445/196688Test

الإتاحة: https://doi.org/10.1182/bloodadvances.2022008028Test
http://hdl.handle.net/2445/196688Test

المؤلفون: Izquierdo, Cristina Pascual, Mingot-Castellano, María Eva, Fuentes, Ana E. Kerguelen, García-Arroba Peinado, José, Cid, Joan, Jimenez, Maria Moraima, Valcarcel, David, Gómez-Seguí, Inés, de la Rubia, Javier, Martin, Paz, Goterris, Rosa, Hernández, Luis, Tallón, Inmaculada, Varea, Sara, Fernández, Marta, García-Muñoz, Nadia, Vara, Míriam, Zarzoso, Miguel Fernández, García-Candel, Faustino, Paciello, María Liz, García-García, Irene, Zalba, Saioa, Campuzano, Verónica, Gala, José María, Estévez, Julia Vidán, Jiménez, Gemma Moreno, López Lorenzo, José Luis, Arias, Elena González, Freiría, Carmen, Solé, María, Ávila Idrovo, Laura Francisca, Hernández Castellet, José Carlos, Cruz, Naylen, Lavilla, Esperanza, Pérez-Montaña, Albert, Atucha, Jon Ander, Moreno Beltrán, María Esperanza, Moreno Macías, Juán Ramón, Salinas, Ramón, del Rio-Garma, Julio

المصدر: Blood Advances; December 2022, Vol. 6 Issue: 24 p6219-6227, 9p

مستخلص: Immune thrombotic thrombocytopenic purpura (iTTP) is a thrombotic microangiopathy caused by anti-ADAMTS13 antibodies. Caplacizumab is approved for adults with an acute episode of iTTP in conjunction with plasma exchange (PEX) and immunosuppression. The objective of this study was to analyze and compare the safety and efficacy of caplacizumab vs the standard of care and assess the effect of the concomitant use of rituximab. A retrospective study from the Spanish TTP Registry of patients treated with caplacizumab vs those who did not receive it was conducted. A total of 155 patients with iTTP (77 caplacizumab, 78 no caplacizumab) were included. Patients initially treated with caplacizumab had fewer exacerbations (4.5% vs 20.5%; P < .05) and less refractoriness (4.5% vs 14.1%; P < .05) than those who were not treated. Time to clinical response was shorter when caplacizumab was used as initial treatment vs caplacizumab used after refractoriness or exacerbation. The multivariate analysis showed that its use in the first 3 days after PEX was associated with a lower number of PEX (odds ratio, 7.5; CI, 2.3-12.7; P < .05) and days of hospitalization (odds ratio, 11.2; CI, 5.6-16.9; P < .001) compared with standard therapy. There was no difference in time to clinical remission in patients treated with caplacizumab compared with the use of rituximab. No severe adverse event was described in the caplacizumab group. In summary, caplacizumab reduced exacerbations and refractoriness compared with standard of care regimens. When administered within the first 3 days after PEX, it also provided a faster clinical response, reducing hospitalization time and the need for PEX.

المؤلفون: Martín, Iván, Villamón, Eva, Abellán, Rosario, Calasanz, Maria José, Irigoyen, Aroa, Sanz, Guillermo, Such, Esperanza, Mora, Elvira, Gutiérrez, Míriam, Collado, Rosa, García‐Serra, Rocío, Vara, Míriam, Blanco, Mª Laura, Oiartzabal, Itziar, Álvarez, Sara, Bernal, Teresa, Granada, Isabel, Xicoy, Blanca, Jerez, Andrés, Calabuig, Marisa

المصدر: British Journal of Haematology; Aug2021, Vol. 194 Issue 4, p708-717, 10p

مصطلحات موضوعية: GENETIC mutation, DELETION mutation, PROGNOSIS, MYELODYSPLASTIC syndromes, AZACITIDINE, OVERALL survival, 22Q11 deletion syndrome

مستخلص: Summary: In myelodysplastic syndromes (MDS), the 20q deletion [del(20q)] may cause deletion of the ASXL1 gene. We studied 153 patients with MDS and del(20q) to assess the incidence, prognostic value and impact on response to azacitidine (AZA) of ASXL1 chromosomal alterations and genetic mutations. Additionally, in vitro assay of the response to AZA in HAP1 (HAP1WT) and HAP1 ASXL1 knockout (HAP1KN) cells was performed. ASXL1 chromosomal alterations were detected in 44 patients (28·5%): 34 patients (22%) with a gene deletion (ASXL1DEL) and 10 patients (6·5%) with additional gene copies. ASXL1DEL was associated with a lower platelet count. The most frequently mutated genes were U2AF1 (16%), ASXL1 (14%), SF3B1 (11%), TP53 (7%) and SRSF2 (6%). ASXL1 alteration due to chromosomal deletion or genetic mutation (ASXL1DEL/ASXL1MUT) was linked by multivariable analysis with shorter overall survival [hazard ratio, (HR) 1·84; 95% confidence interval, (CI): 1·11–3·04; P = 0·018] and a higher rate for acute myeloid leukaemia progression (HR 2·47; 95% CI: 1·07–5·70, P = 0·034). ASXL1DEL/ASXL1MUT patients were correlated by univariable analysis with a worse response to AZA. HAP1KN cells showed more resistance to AZA compared to HAP1WT cells. In conclusion, ASXL1 alteration exerts a negative impact on MDS with del(20q) and could become useful for prognostic risk stratification and treatment decisions. [ABSTRACT FROM AUTHOR]

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المؤلفون: Vara, Míriam, Moreno, Maite, Arzuaga, Javier, Hormaza, Sara, García-Ruiz, Juan Carlos

المصدر: Blood; November 2023, Vol. 142 Issue: 1, Number 1 Supplement 1 p5656-5656, 1p

مستخلص: Paroxysmal Nocturnal Hemoglobinuria (PNH) is characterized by intravascular hemolysis, thrombosis and bone marrow failure. The appearance of the anti-C5 monoclonal antibody eculizumab revolutionized the treatment of PNH, controlling hemolysis and the incidence of thrombosis. However, some patients persist with anemia, due in part to extravascular C3-mediated hemolysis. More recently, the anti-C3 monoclonal antibody pegcetacoplan, which blocks the most proximal part of the complement cascade, has been marketed in Europe, achieving improvements in anemia in these patients. However, intercurrent infectious episodes or surgical interventions can trigger a hemolytic flare, so dose adjustments are necessary to prevent their occurrence. There is hardly any published information or established consensus on how to treat these patients. We present this case as an example of a successful treatment of a patient diagnosed with PNH under treatment with pegcetacoplan who has undergone major surgery.

المؤلفون: Vallejo, Carlos, Bonanad Boix, Santiago, Carnicero, Fernando, Gaya, Anna, Gonzalez, Ana Pilar, Fernandez, Fernando Ataulfo Gonzalez, Lavilla, Esperanza, Salido, Eduardo José, Sanchez Anton, Piva, Vara, Míriam

المصدر: Blood; November 2023, Vol. 142 Issue: 1, Number 1 Supplement 1 p5653-5653, 1p

مستخلص: Introduction: C5 inhibitors (C5i) treatment has dramatically improved the management of intravascular hemolysis (IVH) in PNH patients. However, a significant proportion of patients remain anemic due to suboptimal control of IVH and the development of extravascular hemolysis (EVH), consequence of C5i treatment. Pegcetacoplan (PEG) is the first proximal inhibitor of the complement system, through the inhibition of C3, allowing to control both IVH and EVH. PEG has recently been marketed in Spain for use in adult PNH patients who remain anemic after treatment with an C5i for at least 3 months. The objective of this work is to analyze the response of the first patients treated with PEG in real life in Spain.

المؤلفون: Mingot, Maria Eva, Pascual, Cristina Izquierdo, Garcia-Candel, Faustino, Martínez Nieto, Jorge, García-Arroba Peinado, José, De La Rubia, Javier, Gomez Segui, Ines, Pacielo Coronel, Maria Liz, Valcárcel, David, Jiménez Balarezo, Moraima, Cid, Joan, Lozano, Miquel, Garcia Gala, Jose Maria, Angos Vazquez, Sonia, Vara, Míriam, Guerra, Luisa, Avila Idrobo, Francisca, Oliva Hernandez, ANA Yurena, Zalba, Saoia, Tallon Ruiz, Inmaculada, Ortega Sánchez, Sandra, Goterris, Rosa, Moreno Jimenez, Maria, Dominguez Acosta, Lourdes, Arraiz Ramirez, Maria, Hernández, Luis, Flores Ballesteros, Elena, Del Rio Garma, Julio

المصدر: Blood; November 2023, Vol. 142 Issue: 1, Number 1 Supplement 1 p5394-5394, 1p

مستخلص: INTRODUCTION

المؤلفون: Candía Callirgos, Abraham José, Mamani Vara, Miriam Jessica, Tabraj Esteban, Heidy Stefanny

المصدر: Universidad Tecnológica del Perú ; Repositorio Institucional - UTP

مصطلحات موضوعية: Comercio de frutas, Potencial de exportación, https://purl.org/pe-repo/ocde/ford#5.02.04Test

الوصف: El presente plan de negocios “Exportación de pitahaya amarilla fresca al mercado de Japón – Tokio Metropolitana” tiene como finalidad conocer y determinar los diversos aspectos que se requieren para la exportación de pitahaya al mercado de Tokio-Metropolitana. Par ello el proyecto de negocio presenta 6 capítulos dentro de los cuales, serán analizados a detalle, iniciando con la descripción de nuestra idea de negocio que será la comercialización y exportación de pitahaya amarilla, la misma que cuenta con diversos usos y beneficios, es por ello que su demanda y/o consumo son enfocadas principalmente como fruta fresca, con numerosas propiedades saludables para el cuerpo humano. Debido a los múltiples beneficios que presenta esta agradable fruta trazaremos un plan de marketing para dar a conocer con mayor claridad nuestro producto en un nuevo mercado como es Japón, Tokio Metropolitana. Buscamos una presentación de la fruta totalmente natural para de esta manera lograr inculcar a las personas a consumirla con mayor frecuencia, planteándose la alternativa de consumir un producto netamente original, natural y vitamínico, la misma que con sus beneficios y ventajas les permita reducir los daños continuos a su salud en un futuro. Es aquí donde se encuentra la ventaja competitiva de nuestra empresa y producto, en base a ello determinamos nuestra estrategia de marketing para su comercialización, además de ofrecer una presentación original y llamativa difundiendo así nuestro producto también intencionalmente. Cabe recalcar que la inversión en este proyecto no solo traerá beneficios a sus propietarios y cultivadores, sino que creará nuevas oportunidades para las frutas y productos no tradicionales con efectos multiplicadores para la economía local. ; Trabajo de suficiencia profesional ; Campus Lima Centro

وصف الملف: application/pdf

العلاقة: https://hdl.handle.net/20.500.12867/2298Test

الإتاحة: https://doi.org/20.500.12867/2298Test
https://hdl.handle.net/20.500.12867/2298Test

المؤلفون: Martín, Iván, Villamón, Eva, Abellán, Rosario, Calasanz, Maria Jose, Irigoyen, Aroa, Sanz, Guillermo F, Such, Esperanza, Mora Casterá, Elvira, Gutiérrez, Míriam, Collado, Rosa, Vara, Míriam, Blanco, Laura, Oiartzabal, Itziar, Álvarez, Sara, Bernal del Castillo, Teresa, Granada, Isabel, Xicoy, Blanca, Zamora, Lurdes, Jerez, Andres, Fernández, Raquel, Calabuig, Marisa, García, Francisca, Sanz, Alejandro, Díez, Rosana, Gil, Ángela, de Paz, Raquel, López, Francisca, González, Teresa, Solano, Carlos, Tormo, Mar

المصدر: Blood; November 2020, Vol. 136 Issue: 1, Number 1 Supplement 1 p1-2, 2p

مستخلص: Introduction:The 20q deletion [del(20q)] is a recurrent chromosomal aberration in myelodysplastic syndromes (MDS) and, as a single abnormality, is associated according to the Revised International Prognostic Scoring System (IPSS-R) with a favorable outcome. However, the breakpoint of del(20q) is very heterogeneous and may cause deletion of the ASXL1gene (20q11.21). This gene is an important epigenetic regulator of hematopoiesis and its mutations have been associated in MDS with a shorter overall survival (OS) and a lower response to azacitidine (AZA).