Recomendaciones para el abordaje clínico de pacientes con púrpura trombocitopénica trombótica
| Autor: | Joan Cid, Inés Gómez Seguí, Rosa Goterris Viciedo, Julio del Río-Garma, Jorge M. Nieto, Luis M. Hernández Mateo, Elena Sebastián, A. González, Javier de la Rubia, Ramón Salinas, Teresa Fidalgo, Grupo Español de Aféresis, María Eva Mingot Castellano, David Valcarcel Ferreiras, Faustino García Candel, Cristina Pascual Izquierdo, Aurora Viejo Llorente, Hector Sarmiento Palao |
|---|---|
| Rok vydání: | 2022 |
| Předmět: |
medicine.medical_specialty
Thrombotic microangiopathy business.industry Organ dysfunction Thrombotic thrombocytopenic purpura General Medicine Evidence-based medicine medicine.disease Haemolysis ADAMTS13 hemic and lymphatic diseases medicine Rituximab Caplacizumab medicine.symptom Intensive care medicine business medicine.drug |
| Zdroj: | Medicina Clínica. 158:630.e1-630.e14 |
| ISSN: | 0025-7753 |
| Popis: | Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy (TMA) characterized by the development of microangiopathic haemolytic anaemia, thrombocytopenia, and ischaemic organ dysfunction associated with ADAMTS13 levels lower than 10% in most cases. Recently there have been numerous advances in the field of PTT, new, rapid and accessible techniques capable of quantifying ADAMTS13 activity and inhibitors. The massive sequencing systems facilitate the identification of polymorphisms in the ADAMTS13 gene. In addition, new drugs such as caplacizumab have appeared and relapse prevention strategies are being proposed with the use of rituximab. The existence of TTP patient registries allow a deeper understanding of this disease but the great variability in the diagnosis and treatment makes it necessary to elaborate guidelines that homogenize terminology and clinical practice. The recommendations set out in this document were prepared following the AGREE methodology. The research questions were formulated according to the PICO format. A search of the literature published during the last 10 years was carried out. The recommendations were established by consensus among the entire group, specifying the existing strengths and limitations according to the level of evidence obtained. In conclusion, this document contains recommendations on the management, diagnosis, and treatment of TTP with the ultimate objective of developing guidelines based on the evidence published to date that allow healthcare professionals to optimize TTP treatment. |
| Databáze: | OpenAIRE |
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