A multicenter experience of thrombotic microangiopathies in turkey: the turkish hematologyresearch and education group (threg)-tma01 study
Yükleniyor...
Dosyalar
Tarih
2018
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Pergamon-elsevıer scıence ltd, the boulevard, langford lane, kıdlıngton, oxford ox5 1gb, england
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment.
We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTSI3 activity/antiADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CAHUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE. (C) 2018 Elsevier Ltd. All rights reserved.
Açıklama
Anahtar Kelimeler
Hemolytıc-uremıc syndrome, thrombocytopenıc purpura, adult patıents, dıagnosıs, management, regıstry, ahus, ttp, eculızumab, consensus
Kaynak
Transfusıon and apheresıs scıence
WoS Q Değeri
Scopus Q Değeri
Cilt
57
Sayı
1
Künye
Tekgunduz, E . Yilmaz, M. Erkurt, MA. Kiki, I. Kaya, AH. Kaynar, L. (2018). A multicenter experience of thrombotic microangiopathies in turkey: the turkish hematologyresearch and education group (threg)-tma01 study. Cilt:57. Sayı:1. 27-30ss.
