التفاصيل البيبلوغرافية
| العنوان: |
Incidence, diagnosis, and outcome of immune‐mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura |
| 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 |
| بيانات النشر: |
Wiley |
| سنة النشر: |
2021 |
| المجموعة: |
Wiley Online Library (Open Access Articles via Crossref) |
| 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. |
| نوع الوثيقة: |
article in journal/newspaper |
| اللغة: |
English |
| DOI: |
10.1002/jca.21894 |
| الإتاحة: |
https://doi.org/10.1002/jca.21894Test |
| حقوق: |
http://onlinelibrary.wiley.com/termsAndConditions#vorTest |
| رقم الانضمام: |
edsbas.72379BF5 |
| قاعدة البيانات: |
BASE |
