Incidence, diagnosis, and outcome of immune-mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura
| 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 C, del Rio-Garma J, de la Rubia J, Viejo A, Mingot E, Cid J, Solanich X, Fernandez-Sojo J, Martin-Sanchez J, Hernandez L, Garcia-Gala J, Alonso N, Gonzalez V, Oliva A, Gomez-Segui I, Goterris R, Guerra L, Garcia-Candel F, Fernandez-Docampo M, Antelo M, Salgado-Barreira A, Salinas R, Spanish Apheresis Grp GEA, Spanish Thrombotic Thrombocytopeni |
| المصدر: | JOURNAL OF CLINICAL APHERESIS r-ISABIAL. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica y Sanitaria de Alicante instname r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe |
| سنة النشر: | 2021 |
| مصطلحات موضوعية: | Adult, Pediatrics, medicine.medical_specialty, Exacerbation, Thrombotic thrombocytopenic purpura, ADAMTS13 Protein, 030204 cardiovascular system & hematology, Nationwide survey, 03 medical and health sciences, 0302 clinical medicine, Refractory, Surveys and Questionnaires, Outcome Assessment, Health Care, medicine, Prevalence, Humans, immune‐, mediated thrombotic thrombocytopenic purpura, Registries, Autoantibodies, Retrospective Studies, treatment, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic, business.industry, Incidence (epidemiology), Incidence, Autoantibody, General Medicine, Hematology, medicine.disease, ADAMTS13, Hospitals, Hospitalization, Cross-Sectional Studies, Treatment Outcome, Spain, incidence, business, 030215 immunology, Rare disease |
| 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. |
| تدمد: | 1098-1101 0733-2459 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e128c29620a46b902efd6480c440c8faTest https://pubmed.ncbi.nlm.nih.gov/33780553Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....e128c29620a46b902efd6480c440c8fa |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 10981101 07332459 |
|---|