TY - JOUR
T1 - A multicenter experience of thrombotic microangiopathies in Turkey
T2 - The Turkish Hematology Research and Education Group (ThREG)-TMA01 study
AU - Tekgündüz, Emre
AU - Yılmaz, Mehmet
AU - Erkurt, Mehmet Ali
AU - Kiki, Ilhami
AU - Kaya, Ali Hakan
AU - Kaynar, Leylagul
AU - Alacacioglu, Inci
AU - Cetin, Guven
AU - Ozarslan, Ibrahim
AU - Kuku, Irfan
AU - Sincan, Gulden
AU - Salim, Ozan
AU - Namdaroglu, Sinem
AU - Karakus, Abdullah
AU - Karakus, Volkan
AU - Altuntas, Fevzi
AU - Sari, Ismail
AU - Ozet, Gulsum
AU - Aydogdu, Ismet
AU - Okan, Vahap
AU - Kaya, Emin
AU - Yildirim, Rahsan
AU - Yildizhan, Esra
AU - Ozgur, Gokhan
AU - Ozcebe, Osman Ilhami
AU - Payzin, Bahriye
AU - Akpinar, Seval
AU - Demirkan, Fatih
N1 - Publisher Copyright:
© 2018 Elsevier Ltd
PY - 2018/2
Y1 - 2018/2
N2 - 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 ADAMTS13 activity/anti-ADAMTS13 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/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (175) 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.
AB - 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 ADAMTS13 activity/anti-ADAMTS13 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/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (175) 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.
KW - HUS
KW - Hemolytic-uremic syndrome
KW - TTP
KW - Thrombotic microangiopathy
KW - Thrombotic thrombocytopenic purpura
UR - https://www.scopus.com/pages/publications/85042560693
U2 - 10.1016/j.transci.2018.02.012
DO - 10.1016/j.transci.2018.02.012
M3 - Article
C2 - 29503132
AN - SCOPUS:85042560693
SN - 1473-0502
VL - 57
SP - 27
EP - 30
JO - Transfusion and Apheresis Science
JF - Transfusion and Apheresis Science
IS - 1
ER -