• Medicina clinica · Jun 2022

    Practice Guideline

    Recommendations for the diagnosis and treatment of patients with thrombotic thrombocytopenic purpura.

    • María Eva Mingot Castellano, Cristina Pascual Izquierdo, Ataulfo González, Aurora Viejo Llorente, David Valcarcel Ferreiras, Elena Sebastián, Faustino García Candel, Héctor Sarmiento Palao, Inés Gómez Seguí, Javier de la Rubia, Joan Cid, Jorge Martínez Nieto, Luis Hernández Mateo, Rosa Goterris Viciedo, Teresa Fidalgo, Ramon Salinas, Julio Del Rio-Garma, and Grupo Español de Aféresis (GEA).
    • Servicio de Hematología, Hospital Universitario Virgen del Rocío, Sevilla, España. Electronic address: mariae.mingot.sspa@juntadeandalucia.es.
    • Med Clin (Barc). 2022 Jun 24; 158 (12): 630.e1630.e14630.e1-630.e14.

    AbstractThrombotic 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.Copyright © 2021 Elsevier España, S.L.U. All rights reserved.

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