Guidelines

Vol. 22 No. 2 (2024): Blood Transfusion 2-2024 (March-April)

Diagnosis and clinical management of thrombotic thrombocytopenic purpura (TTP): a consensus statement from the TTP Catalan group

Authors

Key words: ADAMTS13, plasma exchange, thrombotic microangiopathies, thrombotic thrombocytopenic purpura
Publication Date: 2023-09-04

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a low prevalence disease characterized by severe deficiency of the enzyme ADAMTS13, leading to the development of thrombotic microangiopathy (TMA) and often resulting in severe organ disfunction. TTP is an extremely serious condition and, therefore, timely and appropriate treatment is critical to prevent life-threatening complications.
Over the past 25 years, significant advances in the understanding of the pathophysiology of immune TTP have led to the development of readily available techniques for measuring ADAMTS13 levels, as well as new drugs that are particularly effective in the acute phase and in preventing relapses. These developments have improved the course of the disease.
Given the complexity of the disease and its various clinical and laboratory manifestations, early diagnosis and treatment can be challenging.
To address this challenge, a group of experienced professionals from the Catalan TTP group have developed this consensus statement to standardize terminology, diagnosis, treatment and follow up for immune TTP, based on currently available scientific evidence in the field. This guidance document aims to provide healthcare professionals with a comprehensive tool to make more accurate and timely diagnosis of TTP and improve patient outcomes.

Authors

Nadia Garcia Muñoz - emotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary of Bellvitge, L´Hospitalet de Llobregat, Catalonia, Spain https://orcid.org/0000-0003-2753-2261

Sandra Ortega - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary of Bellvitge, L´Hospitalet de Llobregat, Catalonia, Spain

Xavier Solanich - Internal Medicine Dept., Hospital Universitary of Bellvitge, L´Hospitalet de Llobregat, Catalonia, Spain

Cid Joan - Hemotherapy and Hemostasis, ICMHO, Clinic Barcelona, IDIBAPS, University of Barcelona, Catalonia, Spain https://orcid.org/0000-0001-5445-4508

Maribel Díaz - Hemostasis and Erythropathology Pathology Department CDB, Clinic Barcelona, IDIBAPS, University of Barcelona, Barcelona, Catalonia, Spain

Moreno Ana Belen - Hemostasis and Erythropathology Pathology Department CDB, Clinic Barcelona, IDIBAPS, University of Barcelona, Barcelona, Catalonia, Spain

Ancochea Águeda - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary Germans Trias I Pujol, Badalona, Catalonia, Spain

Santos Mireia - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary Germans Trias I Pujol, Badalona, Catalonia, Spain

Hernández Inés - Hemathology, Catalan Institute of Oncology - Hospital Germans Trias i Pujol, Badalona, Catalonia, Spain

Sanchez Juan Manuel - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary Arnau de Vilanova, Lleida, Catalonia, Spain

Luaña Armando - Hemathology, Hospital Universitary Arnau de Vilanova, Lleida, Catalonia, Spain

García Jose - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary de Tarragona Joan XIII, University Rovira i Virgili, Catalonia, Spain

Escoda Lourdes - Hemathology, Catalan Institute of Oncology Tarragona Hospital Universitary de Tarragona Joan XIII, University Rovira i Virgili, Catalonia, Spain https://orcid.org/0000-0003-3288-9716

Medina Laura - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital de la Santa Creu i Sant Pau, Catalonia, Spain

Ferrer Gonzalo José - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital de la Santa Creu i Sant Pau, Catalonia, Spain

López Jordi - Hemathology, Hospital de la Santa Creu i Sant Pau, Catalonia, Spain

Céspedes Roberto - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Doctor Josep Trueta, Girona, Catalonia, Spain https://orcid.org/0000-0002-3240-0266

Díaz Johana Alejandra - Hemathology, Catalan Institute of Oncology, Hospital Doctor Josep Trueta, Girona, Catalonia, Spain

Pons Verónica - Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Hospital Universitary de la Vall d’Hebron, Catalonia, Spain

Valcárcel David - Hemathology, VHIO, Hospital Universitary de la Vall d’Hebron, Catalonia, Spain

Grifols Joan Ramon - Medical Direction, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cel·lular i Tissular, Barcelona, Catalonia, Spain

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