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A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study |
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| Authors: | Emre Tekgündüz Mehmet Yılmaz Mehmet Ali Erkurt Ilhami Kiki Ali Hakan Kaya Leylagul Kaynar Inci Alacacioglu Guven Cetin Ibrahim Ozarslan Irfan Kuku Gulden Sincan Ozan Salim Sinem Namdaroglu Abdullah Karakus Volkan Karakus Fevzi Altuntas Ismail Sari Gulsum Ozet Fatih Demirkan |
| Institution: | 1. Ankara Oncology Hospital, Hematology and BMT Clinic, Ankara, Turkey;2. Gaziantep University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Gaziantep, Turkey;3. Inonu University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Malatya, Turkey;4. Erzurum University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Erzurum, Turkey;5. Erciyes University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Kayseri, Turkey;6. Dokuz Eylul University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Izmir, Turkey;7. Bezmialem University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Istanbul, Turkey;8. Akdeniz University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Antalya, Turkey;9. Bozyaka Education and Research Hospital, Hematology Clinic, Izmir, Turkey;10. Dicle University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Diyarbakir, Turkey;11. Mugla Sitki Kocman University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Mugla, Turkey;12. Pamukkale University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Denizli, Turkey;13. Ankara Numune Education and Research Hospital, Hematology and BMT Clinic, Ankara, Turkey;14. Celal Bayar University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Manisa, Turkey;15. Gulhane Military Medical Academy University, Department of Internal Medicine, Division of Hematology, Ankara, Turkey;p. Hacettepe University, Faculty of Medicine, Department of Internal Medicine, Division of Hematology, Ankara, Turkey;q. Katip Celebi University, Ataturk Training and Research Hospital, Hematology Clinic, Izmir, Turkey;r. Sisli Hamidiye Etfal Education and Research Hospital, Hematology Clinic, Istanbul, Turkey |
| Abstract: | 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. |
| Keywords: | Thrombotic microangiopathy Thrombotic thrombocytopenic purpura Hemolytic-uremic syndrome TTP HUS |
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