المؤلفون: Serrano Simonneau, David, Pascual Izquierdo, Cristina, Fundación MAPFRE. Área de Promoción de la Salud

الوصول الحر: http://europeana.eu/portal/record/2022701/oai_documentacion_mapfre_es_154803.htmlTest

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: Mingot-Castellano, María Eva, Canaro, Mariana, Sánchez-González, Blanca, Álvarez-Román, María Teresa, Bárez-García, Abelardo, Bernardo-Gutiérrez, Ángel, Bernat-Pablo, Silvia, Bolaños-Calderón, Estefanía, Butta, Nora, Caballero-Navarro, Gonzalo, Caparrós-Miranda, Isabel Socorro, Entrena-Ureña, Laura, Fernández-Fuertes, Luis Fernando, García-Frade, Luis Javier, Gómez del Castillo Solano, María del Carmen, González-López, Tomás José, Grande-García, Carlos, Guinea de Castro, José María, Jarque, Isidro, Jiménez-Bárcenas, Reyes, López-Ansoar, Elsa, Martínez-Carballeira, Daniel, Martínez-Robles, Violeta, Monteagudo-Montesinos, Emilio, Páramo-Fernández, José Antonio, Perera-Álvarez, María del Mar, Soto-Ortega, Inmaculada, Valcárcel, David, Pascual-Izquierdo, Cristina, Spanish Immune Thrombocytopenia Group (GEPTI), Group of Immune Thrombocytopenia of the Spanish Society of Paediatric Haematology Oncology (SEHOP)

مصطلحات موضوعية: Primary immune thrombocytopenia, Glucocorticoids, Intravenous immunoglobulins, Fostamatinib, Rituximab, Recommendations

الوصف: © 2023 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0Test/). ; Primary immune thrombocytopenia (ITP) is a complex autoimmune disease whose hallmark is a deregulation of cellular and humoral immunity leading to increased destruction and reduced production of platelets. The heterogeneity of presentation and clinical course hampers personalized approaches for diagnosis and management. In 2021, the Spanish ITP Group (GEPTI) of the Spanish Society of Hematology and Hemotherapy (SEHH) updated a consensus document that had been launched in 2011. The updated guidelines have been the reference for the diagnosis and management of primary ITP in Spain ever since. Nevertheless, the emergence of new tools and strategies makes it advisable to review them again. For this reason, we have updated the main recommendations appropriately. Our aim is to provide a practical tool to facilitate the integral management of all aspects of primary ITP management. ; Peer reviewed

وصف الملف: application/pdf

العلاقة: Publisher's version; The underlying dataset has been published as supplementary material of the article in the publisher platform at https://doi.org/10.3390/jcm12206422Test; https://doi.org/10.3390/jcm12206422Test; Sí; Journal of Clinical Medicine 12(20): 6422 (2023); http://hdl.handle.net/10261/353856Test

الإتاحة: https://doi.org/10.3390/jcm12206422Test
http://hdl.handle.net/10261/353856Test

المؤلفون: Scully, Marie, Baptista, Jovanna, Bhattacharya, Indranil, Cataland, Spero, Coppo, Paul, Cuccia, Loredana, Dutt, Tina, Susan Huang, Shih-Han, Pascual Izquierdo, Cristina, Eva Mingot-Castellano, María, Parnes, Aric, Pavenski, Katerina, Rajavel, Kavitha, Jarque, Isidro, Wang, Linda T., Fernández Zarzoso, Miguel, Zhu, Andy, Mellgård, Björn

المصدر: HemaSphere ; volume 7, issue S3, page e8651306 ; ISSN 2572-9241

الإتاحة: https://doi.org/10.1097/01.hs9.0000968132.86513.06Test

المؤلفون: Mingot-Castellano, María Eva, Butta, Nora, Canaro, Mariana, Gómez del Castillo Solano, María del Carmen, Sánchez-González, Blanca, Jiménez-Bárcenas, Reyes, Pascual-Izquierdo, Cristina, Caballero-Navarro, Gonzalo, Entrena Ureña, Laura, González-López, Tomás J.

المساهمون: Butta, Nora, Pascual-Izquierdo, Cristina

مصطلحات موضوعية: COVID-19, Vaccines, ITP, VITT, TTP, AIHA and Evans syndrome, Antiphospholipid syndrome, Catastrophic antiphospholipid syndrome

الوصف: Worldwide vaccination against SARS-CoV-2 has allowed the detection of hematologic autoimmune complications. Adverse events (AEs) of this nature had been previously observed in association with other vaccines. The underlying mechanisms are not totally understood, although mimicry between viral and self-antigens plays a relevant role. It is important to remark that, although the incidence of these AEs is extremely low, their evolution may lead to life-threatening scenarios if treatment is not readily initiated. Hematologic autoimmune AEs have been associated with both mRNA and adenoviral vector-based SARS-CoV-2 vaccines. The main reported entities are secondary immune thrombocytopenia, immune thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia, Evans syndrome, and a newly described disorder, so-called vaccine-induced immune thrombotic thrombocytopenia (VITT). The hallmark of VITT is the presence of anti-platelet factor 4 autoantibodies able to trigger platelet activation. Patients with VITT present with thrombocytopenia and may develop thrombosis in unusual locations such as cerebral beds. The management of hematologic autoimmune AEs does not differ significantly from that of these disorders in a non-vaccine context, thus addressing autoantibody production and bleeding/thromboembolic risk. This means that clinicians must be aware of their distinctive signs in order to diagnose them and initiate treatment as soon as possible. ; Peer reviewed

العلاقة: Publisher's version; https://doi.org/10.3390/vaccines10060961Test; Sí; Vaccines 10(6): 961 (2022); http://hdl.handle.net/10261/280457Test

الإتاحة: https://doi.org/10.3390/vaccines10060961Test
http://hdl.handle.net/10261/280457Test

المؤلفون: Mingot-Castellano, María-Eva, García-Candel, Faustino, Martínez-Nieto, Jorge, García-Arroba, José, de la Rubia-Comos, Javier, Gómez-Seguí, Inés, Paciello-Coronel, María-Liz, Valcárcel-Ferreiras, David, Jiménez, Moraima, Cid, Joan, Lozano, Miquel, García-Gala, José-María, Angós-Vazquez, Sonia, Vara-Pampliega, Miriam, Guerra-Domínguez, Luisa, Ávila-Idrobo, Laura-Francisca, Oliva-Hernandez, Ana, Zalba-Marcos, Saioa, Tallón-Ruiz, Inmaculada, Ortega-Sánchez, Sandra, Goterris-Viciedo, Rosa, Moreno-Jiménez, Gemma, Domínguez-Acosta, Lourdes, Araiz-Ramírez, María, Hernández-Mateos, Luis, Flores-Ballesteros, Elena, del Río-Garma, Julio, Pascual-Izquierdo, Cristina, Abio Calvete, Mariola, Albert, Albert, Alberto López García, Alberto, Alegre, Adrian, Alkorta Eizagirre, Aitziber, Alonso Escobar, María Nieves, Alonso Madrigal, Cristina, Amunarriz, Cristina, Antelo Caamaño, María Luisa, Arbona Castaño, Cristina, Ballester Ruiz, Maria Carmen, Ballina Martín, Belén, Berberana Fernández de Murias, Margarita, Berrueco Moreno, Ruben, Bueno, Jose Luis, Calderón López, María Teresa, Chica Gullón, Esther, Cid Vidal, Joan, Contreras Barbeta, Enric, Cuéllar Pérez-Ávila, Clara, de la Rubia Comos, Javier, Del Orbe Barreto, Rafael Andres, Del Río Garma, Julio, Díaz Valdés, José R., Diaz-Ricart, Maribel, Diez Gallarreta, Zuriñe, Dueñas Hernando, Virginia, Eguia, Blanca, Escoda, Lourdes, Fernández Docampo, Marta, Fernandez Fuertes, Fernando, Fernández Muñoz, Hermogenes, Fernández Sánchez de Mora, Maria Carmen, Fernandez Zarzoso, Miguel, Fidalgo, Teresa, Flores Ballester, Elena, Fonte Feal, Cristina, Galvez, Francisco Javier, Garcia Arroba Peinado, Jose, García Candel, Faustino, Garcia Erce, Jose Antonio, García Gala, José María, Gimeno, JJ, Gómez, Delia, Gómez Seguí, Inés, Gomez Vazquez, Maria Jesus, Gonzalez, Carlos, González Fernández, Fernando Ataulfo, Gonzalez Porras, Jose Ramon, Gonzalez Rodriguez, Victoria Paz, Goterris Viciedo, Rosa, Guerra Domínguez, Luisa, Guillén García, Helga, Hernandez, Adoracion, Hernández Castellet, José Carlos, Hernandez Mohedo, Francisca, Hernandez Vazquez, Laura, Hidalgo Soto, Marta, Hong Tam, Azueg Hang, Kerguelen Fuentes, Ana Lilia, Leal Bento, Marta, Lopes, Raquel, López, Olga, López Chuliá, Francisca, Maria Jose Busto, Maria Jose, Martín Hernández, María Paz, Martinez Estefano, Elvira, Martinez Nieto, Jorge, Martinez Redondo, Consuelo, Martinez Revuelta, Eva, Medina Marrero, Laura, Mingot Castellano, María Eva, Morales Sanz, María Dolores, Moreno, Gemma, Moreno Beltrán, Mª Esperanza, Moreno Chulilla, Jose Antonio, Nistal Gil, Sara, Oliva Hernandez, Ana Yurena, Pascual, Teresa, Pascual Izquierdo, Crisina, Paumard Rodríguez, Elena, Pecos, Patricia, Peña Marcos, Francisco, Pereira Coelho, Daniela Sofia, Pérez Segura, Gloria Maria, Perez-Lopez, Olga, Prieto Pareja, Elena, Ramiro, Laia, Richart López, Luis Alberto, Rodriguez Dominguez, Maria Jesus, Rodriguez Nuñez, Antonio, Ruiz Sainz, María Elena, Saez Serrano, Isabel, Salinas Argente, Ramón, Sanchez, Maria Elena, Sanchez Anton, Piva, Sánchez Fernández, Mª Soledad, Sebastian, Elena, Simona, Gabriela, Solanich Moreno, Xabier, Soledad Casado, Soledad, Tallón, Jose David, Turcu, Violeta, Valledor Méndez, Manuel, Vidan Y Estevez, Julia, Viejo Llorente, Aurora, Bienert, Álvaro, Serrano, Alfons, Llorente, Laura, Campuzano, Verónica, Tallón, Inmaculada, Pons, Verónica, Linares, Mónica, Valles, Ana, Martínez Francés, Antonio, Freiria, Carmen, González Arias, Elena, Araujo, Enmanuel, Marco de Lucas, Fernando, López, Juan Antonio, Uribe Barrientos, Marisol, Calviño, Michael, Gómez Calafat, Montse, Marco Vera, Pascual, Fariña, Sabela, Zalba, Saioa, Monsalvo, Silvia, Escamilla, Virginia

المصدر: Blood; May 2024, Vol. 143 Issue: 18 p1807-1815, 9p

مستخلص: •There is no delay in ADAMTS13 recovery after PEX start in caplacizumab–treated patients with iTTP from the Spanish registry.•Caplacizumab allows suspending PEX earlier, thus creating the impression that there is a delay in ADAMTS13 recovery after PEX end.

المؤلفون: Meijon-Ortigueira, Maria del Mar, Solares, Isabel, Muñoz-Delgado, Cecilia, Stanescu, Sinziana, Morado, Marta, Pascual-Izquierdo, Cristina, Villalon Blanco, Lucía, Belanger Quintana, Amaya, Menéndez-Conde, Covadonga Pérez, Morales-Conejo, Montserrat, Villarrubia-Espinosa, Jesús

المصدر: Biomedicines; Mar2024, Vol. 12 Issue 3, p579, 10p

مصطلحات موضوعية: GAUCHER'S disease, KIDNEY tumors, ENZYME deficiency, GENETIC counseling, GENETIC disorders

مستخلص: Gaucher disease is an inherited disorder in which there is a deficiency of the enzyme glucocerebrosidase, which leads to the accumulation of glucosylceramide. Although much scientific evidence is now available, there is still limited data on the impact on the different life stages of women with this disease. Among other alterations, a delay in menarche has been described, although it has not been related to fertility problems. Menorrhagia is relatively frequent, being related to the presence of thrombocytopenia, thrombocytopathies or coagulation disorders. On the other hand, pregnancy planning is an increasingly frequent concern. All patients should undergo genetic counseling, and it is important to monitor the appearance or worsening of organomegaly, bone and hematologic abnormalities to establish clinical and therapeutic recommendations. Management during the puerperium will depend on the evolution of gestation, and, during the lactation period, the potential appearance of bone complications should be assessed. An early onset of menopause, compared to the general population, has also been described, which may accelerate the development of osteopenia. Finally, although the usual screening protocols for neoplasms are currently being performed, it is recommended to watch for early signs of liver or renal neoplasms when examining the results of imaging tests performed during evaluations for this disease. [ABSTRACT FROM AUTHOR]

: Copyright of Biomedicines is the property of MDPI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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: Mingot-Castellano, María Eva, Bastida, José María, Caballero-Navarro, Gonzalo, Entrena Ureña, Laura, González-López, Tomás José, González-Porras, José Ramón, Butta, Nora, Canaro, Mariana, Jiménez-Bárcenas, Reyes, Gómez Del Castillo Solano, María Del Carmen, Sánchez-González, Blanca, Pascual-Izquierdo, Cristina, On Behalf Of The Gepti

مصطلحات موضوعية: autoantibodies, immune thrombocytopenia, platelets, targeted therapies, thrombopoietin

الوصف: Primary immune thrombocytopenia (ITP) is an autoimmune disorder that causes low platelet counts and subsequent bleeding risk. Although current corticosteroid-based ITP therapies are able to improve platelet counts, up to 70% of subjects with an ITP diagnosis do not achieve a sustained clinical response in the absence of treatment, thus requiring a second-line therapy option as well as additional care to prevent bleeding. Less than 40% of patients treated with thrombopoietin analogs, 60% of those treated with splenectomy, and 20% or fewer of those treated with rituximab or fostamatinib reach sustained remission in the absence of treatment. Therefore, optimizing therapeutic options for ITP management is mandatory. The pathophysiology of ITP is complex and involves several mechanisms that are apparently unrelated. These include the clearance of autoantibody-coated platelets by splenic macrophages or by the complement system, hepatic desialylated platelet destruction, and the inhibition of platelet production from megakaryocytes. The number of pathways involved may challenge treatment, but, at the same time, offer the possibility of unveiling a variety of new targets as the knowledge of the involved mechanisms progresses. The aim of this work, after revising the limitations of the current treatments, is to perform a thorough review of the mechanisms of action, pharmacokinetics/pharmacodynamics, efficacy, safety, and development stage of the novel ITP therapies under investigation. Hopefully, several of the options included herein may allow us to personalize ITP management according to the needs of each patient in the near future.

وصف الملف: application/pdf

العلاقة: http://hdl.handle.net/10668/21535Test; PMC9318546; https://www.mdpi.com/1424-8247/15/7/779/pdf?version=1656379336Test; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9318546/pdfTest

الإتاحة: https://doi.org/10.3390/ph15070779Test
http://hdl.handle.net/10668/21535Test
https://www.mdpi.com/1424-8247/15/7/779/pdf?version=1656379336Test
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9318546/pdfTest

المؤلفون: González-López, Tomás José, Bárez, Abelardo, Bernardo-Gutiérrez, Angel, Bernat, Silvia, Canaro-Hirnyk, Mariana, Entrena-Ureña, Laura, Fernández-Fuertes, Fernando, Guinea de Castro, José María, Jiménez-Bárcenas, Reyes, Pascual-Izquierdo, Cristina, Sánchez-González, Blanca, Jarque, Isidro

المصدر: Infectious Diseases and Therapy ; volume 12, issue 2, page 303-315 ; ISSN 2193-8229 2193-6382

مصطلحات موضوعية: Infectious Diseases, Microbiology (medical)

الإتاحة: https://doi.org/10.1007/s40121-022-00745-2Test