Prospective registry of adult patients receiving therapeutic plasma exchange with a presumptive diagnosis of thrombotic microangiopathy (TMA) : The Turkish hematology research and education group (ThREG)-TMA02 study

Copyright © 2021 Elsevier Ltd. All rights reserved..

Thrombotic microangiopathy(TMA) is a pathological diagnosis characterized by abnormalities of small vessels leading to microvascular thrombosis of arterioles and capillaries. The current prospective, non-interventional, multicenter (n:18) study aimed to define distribution of different TMA forms in adult Turkish patients who were referred for therapeutic plasma exchange (TPE) for a presumptive diagnosis of TMA. Patients with serum ADAMTS13 activity <5% were diagnosed as acquired thrombotic thrombocytopenic purpura (aTTP). Patients presenting with ADAMTS13 activity 6-10 % / normal renal function and patients with ADAMTS13 activity >10 %, normal renal function and no secondary TMA were treated as unclassified TMA. The study included a total of 97 patients (female: 60; male: 30) with a median age of 48 (18-74). Detailed evaluation at 1 month after hospital admission revealed aTTP, secondary TMA, infection/complement-associated hemolytic uremic syndrome and unclassified TMA in 32 (33 %), 33 (34 %), 26 (27 %) and 6 (6%) patients respectively. As subclassification of various TMAs will dictate specific therapy, proper diagnosis in a timely manner is of utmost clinical significance.

Medienart:

E-Artikel

Erscheinungsjahr:

2021

Erschienen:

2021

Enthalten in:

Zur Gesamtaufnahme - year:2021

Enthalten in:

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis - (2021) vom: 16. Nov., Seite 103312

Sprache:

Englisch

Beteiligte Personen:

Akpınar, Seval [VerfasserIn]
Tekgunduz, Emre [VerfasserIn]
Erkurt, Mehmet Ali [VerfasserIn]
Esen, Ramazan [VerfasserIn]
Yılmaz, Mehmet [VerfasserIn]
Karakus, Volkan [VerfasserIn]
Vural, Filiz [VerfasserIn]
Gediz, Fusun [VerfasserIn]
Aydogdu, Ismet [VerfasserIn]
Kaynar, Leylagul [VerfasserIn]
Korkmaz, Serdal [VerfasserIn]
Goker, Hakan [VerfasserIn]
Kelkitli, Engin [VerfasserIn]
Ayyıldız, Orhan [VerfasserIn]
Demirkan, Fatih [VerfasserIn]

Links:

Volltext

Themen:

Hematopoietic cell transplantation
Hemolytic uremic syndrome
Journal Article
Thrombotic microangiopathy
Thrombotic thrombocytopenic purpura

Anmerkungen:

Date Revised 20.02.2024

published: Print-Electronic

Citation Status Publisher

doi:

10.1016/j.transci.2021.103312

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM333366042