A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education Group (ThREG)-TMA01 study
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Date
2018
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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. © 2018 Elsevier Ltd
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Keywords
ADAMTS13 Protein , Adolescent , Adult , Aged , Aged, 80 and over , Autoantibodies , Female , Follow-Up Studies , Hemolytic-Uremic Syndrome , Humans , Male , Middle Aged , Plasma Exchange , Retrospective Studies , Turkey , eculizumab , immunosuppressive agent , rituximab , steroid , von Willebrand factor cleaving proteinase , ADAMTS13 protein, human , autoantibody , von Willebrand factor cleaving proteinase , adolescent , adult , aged , Article , cohort analysis , controlled study , disease exacerbation , enzyme activity , enzyme blood level , female , follow up , hemolytic uremic syndrome , hospital admission , human , immunosuppressive treatment , kidney failure , major clinical study , male , pathophysiology , plasma exchange , plasma volume , relapse , remission , retrospective study , steroid therapy , thrombotic thrombocytopenic purpura , treatment response , Turkey (republic) , blood , clinical trial , hemolytic uremic syndrome , immunology , middle aged , mortality , multicenter study , pathology , plasma exchange , turkey (bird) , very elderly