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.
References
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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
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
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
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
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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