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


Por: Muñoz NG, Ortega S, Solanich X, Cid J, Díaz M, Moreno AB, Ancochea Á, Santos M, Hernández I, Sanchez JM, Luaña A, García J, Escoda L, Medina L, Ferrer GJ, López J, Céspedes R, Díaz JA, Pons V, Valcárcel D, Grifols JR

Publicada: 1 mar 2024 Ahead of Print: 4 sep 2023
Resumen:
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.

Filiaciones:
Muñoz NG:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary of Bellvitge, L'Hospitalet de Llobregat, Catalonia, Spain

Ortega S:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary of Bellvitge, L'Hospitalet de Llobregat, Catalonia, Spain

Solanich X:
 Internal Medicine, Hospital Universitary of Bellvitge, L'Hospitalet de Llobregat, Catalonia, Spain

Cid J:
 Hemotherapy and Hemostasis, ICMHO, Clinic Barcelona, IDIBAPS, University of Barcelona, Barcelona, Catalonia, Spain

Díaz M:
 Hemostasis and Erythropathology Pathology Department CDB, Clinic Barcelona, IDIBAPS, University of Barcelona, Barcelona, Catalonia, Spain

Moreno AB:
 Hemostasis and Erythropathology Pathology Department CDB, Clinic Barcelona, IDIBAPS, University of Barcelona, Barcelona, Catalonia, Spain

Ancochea Á:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary Germans Trias I Pujol, Badalona, Catalonia, Spain

Santos M:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary Germans Trias I Pujol, Badalona, Catalonia, Spain

Hernández I:
 Hemathology, Catalan Institute of Oncology, Hospital Germans Trias i Pujol, Badalona, Catalonia, Spain

Sanchez JM:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary Arnau de Vilanova, Lleida, Catalonia, Spain

Luaña A:
 Hemathology, Hospital Universitary Arnau de Vilanova, Lleida, Catalonia, Spain

García J:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary de Tarragona Joan XIII, University Rovira i Virgili, Catalonia, Spain

Escoda L:
 Hemathology, Catalan Institute of Oncology Tarragona Hospital Universitary de Tarragona Joan XIII, University Rovira i Virgili, Tarragona, Catalonia, Spain

Medina L:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital de la Santa Creu i Sant Pau, Barcelona, Catalonia, Spain

Ferrer GJ:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital de la Santa Creu i Sant Pau, Barcelona, Catalonia, Spain

López J:
 Hemathology, Hospital de la Santa Creu i Sant Pau, Barcelona, Catalonia, Spain

Céspedes R:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Doctor Josep Trueta, Girona, Catalonia, Spain

Díaz JA:
 Hemathology, Catalan Institute of Oncology, Hospital Doctor Josep Trueta, Girona, Catalonia, Spain

Pons V:
 Hemotherapy, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Hospital Universitary de la Vall d'Hebron, Barcelona, Catalonia, Spain

Valcárcel D:
 Hemathology, VHIO, Hospital Universitary de la Vall d'Hebron, Barcelona, Catalonia, Spain

Grifols JR:
 Medical Direction, Banc de Sang i Teixits, Càtedra UAB de Medicina Transfusional i Teràpia Cellular i Tissular, Barcelona, Catalonia, Spain
ISSN: 17232007





Blood Transfusion
Editorial
SIMTIPRO SRL, VIA DESIDERIO 21, MILAN, 20131, ITALY, Italia
Tipo de documento: Article
Volumen: 22 Número: 2
Páginas: 176-184
WOS Id: 001227816800010
ID de PubMed: 37677097
imagen Open Access

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