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, Cristina, Mingot-Castellano, María Eva, Kerguelen Fuentes, Ana E., García-Arroba, José, Cid, Joan, Jimenez, Moraima, Valcárcel, 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, Vidan, Julia, Moreno-Jiménez, Gemma, López Lorenzo, José Luis, González Arias, Elena, Freiría, Carmen, Solé, María, Ãvila Idrovo, Laura Francisca, Hernández Castellet, José Carlos, Cruz, Naylen, Lavilla, Esperanza, Pérez-Montaña, Albert, Atucha, Jon Ander, Moreno Beltrán, María Esperanza, Moreno Macías, Juan Ramón, Salinas, Ramón, del Rio-Garma, Julio

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: application/pdf

العلاقة: Blood advances; Vol. 6 Núm. 24 (December 2022), p. 6219-6227; https://ddd.uab.cat/record/282297Test; urn:10.1182/bloodadvances.2022008028; urn:oai:ddd.uab.cat:282297; urn:scopus_id:85141669213; urn:articleid:24739537v6n24p6219; urn:pmid:35930694; urn:pmc-uid:9792393; urn:pmcid:PMC9792393; urn:oai:pubmedcentral.nih.gov:9792393

الإتاحة: https://ddd.uab.cat/record/282297Test

المؤلفون: Mingot-Castellano, María Eva, Izquierdo, Cristina Pascual, del Rio Garma, Julio

المصدر: Transfusion and Apheresis Science ; volume 62, issue 3, page 103725 ; ISSN 1473-0502

مصطلحات موضوعية: Hematology

الإتاحة: https://doi.org/10.1016/j.transci.2023.103725Test
https://api.elsevier.com/content/article/PII:S147305022300099X?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S147305022300099X?httpAccept=text/plainTest

المؤلفون: Mingot Castellano, María Eva, Pascual Izquierdo, Cristina, González, Ataulfo, Viejo Llorente, Aurora, Valcarcel Ferreiras, David, Sebastián, Elena, García Candel, Faustino, Sarmiento Palao, Héctor, Gómez Seguí, Inés, de la Rubia, Javier, Cid, Joan, Martínez Nieto, Jorge, Hernández Mateo, Luis, Goterris Viciedo, Rosa, Fidalgo, Teresa, Salinas, Ramon, del Rio-Garma, Julio

المصدر: Medicina Clínica ; volume 158, issue 12, page 630.e1-630.e14 ; ISSN 0025-7753

مصطلحات موضوعية: General Medicine

الإتاحة: https://doi.org/10.1016/j.medcli.2021.03.040Test
https://api.elsevier.com/content/article/PII:S0025775321003328?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S0025775321003328?httpAccept=text/plainTest

المؤلفون: Contreras, Enric, de la Rubia, Javier, del Río-Garma, Julio, Díaz-Ricart, Maribel, García-Gala, José María, Lozano, Miguel

المصدر: Medicina Clínica ; volume 144, issue 7, page 331.e1-331.e13 ; ISSN 0025-7753

مصطلحات موضوعية: General Medicine

الإتاحة: https://doi.org/10.1016/j.medcli.2014.09.013Test
https://api.elsevier.com/content/article/PII:S0025775314007362?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S0025775314007362?httpAccept=text/plainTest

المؤلفون: Pascual‐Izquierdo, Cristina, del Rio‐Garma, Julio, de la Rubia, Javier, Viejo, Aurora, Mingot, Eva, Cid, Joan, Solanich, Xavier, Fernández‐Sojo, Jesús, Martín‐Sánchez, Jesús, Hernández, Luis, García‐Gala, José María, Alonso, Nieves, González, Victoria, Oliva, Ana, Gómez‐Seguí, Inés, Goterris, Rosa, Guerra, Luisa, García‐Candel, Faustino, Fernández‐Docampo, Marta, Antelo, María Luisa, Salgado‐Barreira, Ángel, Salinas, Ramón

المصدر: Journal of Clinical Apheresis ; volume 36, issue 4, page 563-573 ; ISSN 0733-2459 1098-1101

الوصف: Background Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a rare disease characterized by the presence of anti‐ADAMTS13 autoantibodies. Achieving accurate information on incidence and customary disease management is important to provide appropriate diagnostic and therapeutic resources. The aim of this study was to determine the incidence and outcomes of iTTP in Spain. Study design and methods A cross‐sectional survey was carried out among Spanish hospitals, focused on iTTP patients ≥16 years old attended between 2015 and 2017, and those at follow‐up before that interval. Incidence, prevalence, mortality, refractoriness, exacerbations, treatment complications, relapses, and sequelae were estimated. Results Forty‐two hospitals covering roughly 20 million inhabitants answered the survey and reported 203 episodes (138 newly‐diagnosed and 65 relapses), of which 193 (95.1%) were treated. Incidence was 2.67 (95% CI 1.90‐3.45) patients per million inhabitants per year and prevalence 21.44 (95% CI% 19.10‐23.73) patients per million inhabitants. At diagnosis, ADAMTS13 activity and anti‐ADAMTS13 autoantibody were measured in 97% and 84.3% of reported episodes, respectively. Fifteen patients (7.4%) died as a direct consequence of iTTP, 6 of them before receiving any iTTP‐specific treatment. Thirty‐one (16.1%) of the 193 treated episodes were refractory to plasma exchange and corticosteroids, and 51 (26.4%) suffered at least one exacerbation. Conclusion iTTP incidence and prevalence were somewhat higher than those documented in neighboring countries. Together with data on treatments and outcomes, this information will allow us to better estimate what is needed to improve diagnosis and prognosis of iTTP patients in Spain.

الإتاحة: https://doi.org/10.1002/jca.21894Test

المؤلفون: del Río-Garma, Julio, Bobillo, Sabela, de la Rubia, Javier, Pascual, Cristina, García-Candel, Faustino, García-Gala, Jose M., Gonzalez, Reyes, Abril, Laura, Vidan, Julia, Gomez, Maria Jesús, Peña, Francisco, Arbona, Cristina, Martín-Sanchez, Jesús, Moreno, Gemma, Romón, Iñigo, Viejo, Aurora, Oliva, Ana, Linares, Mónica, Salinas, Ramón, Pérez, Sonia, Garcia-Erce, Jose A., Pereira, Arturo

المصدر: Annals of Hematology ; volume 101, issue 1, page 59-67 ; ISSN 0939-5555 1432-0584

مصطلحات موضوعية: Hematology, General Medicine

الإتاحة: https://doi.org/10.1007/s00277-021-04685-8Test

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: Del Río Garma, Julio

العلاقة: http://hdl.handle.net/20.500.11940/6797Test; 11304

الإتاحة: https://doi.org/20.500.11940/6797Test
https://doi.org/10.1016/j.medcli.2010.02.011Test
https://hdl.handle.net/20.500.11940/6797Test

المؤلفون: Pereira, Arturo, Del Río Garma, Julio, García-Erce, José A.

مصطلحات موضوعية: Methylene Blue, Humans, Plasma, azul de metileno, humanos, Costs and Cost Analysis, Quarantine, Spain, AS Ourense, CHUO, AS Santiago

العلاقة: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343590/pdf/blt-17-083.pdfTest; https://www.ncbi.nlm.nih.gov/pubmed/29757136Test; http://hdl.handle.net/20.500.11940/15005Test; 28925

الإتاحة: https://doi.org/20.500.11940/15005Test
https://doi.org/10.2450/2018.0056-18Test
https://hdl.handle.net/20.500.11940/15005Test
https://www.ncbi.nlm.nih.gov/pubmed/29757136Test