The retrospective evaluation of etyology of the patients with thrombotic microanjopathy and the role in plasma variation in treatment in Turkey faculty of Medicine Turgut Özal Medical center hematology

INTRODUCTION Thrombotic Mcroangiopathy (TMA); microangiopathic hemolytic anemia (MAHA) is a disease group characterized by ischemic tissue damage caused by thrombocytopenia and microthrombi. The microthromboses in TMA come from platelet aggregates. Presence of schistocytes in peripheral blood smear, laboratory hematological anemia (non-immunohemolytic anemia, increased lactate dehydrogenase, indirect bilirubin increase, decreased haptoglobulin and reticulocytosis) and normal routine coagulation parameters support TMA diagnosis. TMA is divided into two groups, primer and secondary. In the primer group; Thrombotic thrombocytopenic purpura (TTP) and Hemolytic Uremic Syndrome (HUS) were observed in the second group; pregnancy, autoimmune diseases, malignancy, bone marrow transplantation and the use of certain medicines. Plasmapheresis is the process of separating the patient's plasma and replacing it with replacement liquids such as fresh frozen plasma, albumin, cryoprecipitate, hydroxyethylstarch. Therapeutic plasma exchange (TPD) is the process of therapeutic substitution of the plasmid in which allogeneic plasma is used as the replacement fluid. In our study, we aimed to make an etiological classification in the patient group who received TMA diagnosis with thrombocytopenia and MAHA in our hospital and evaluate TPD's effect on the improvement of laboratory parameters and mortality. METHOD In our study, between 2009-2017, in Inonu University Medicine Faculty Hematology Clinic, 85 patients with TMA diagnosed with laboratory and peripheral blood smear examined retrospectively. TPD treatment was performed by attaching a two-way dialysis catheter to the patient. Complete blood count and biochemical parameters were examined before and after TPD treatment. BULGULAR In the etiological classification of TMA, in our region, HELLP syndrome was found the most frequently, second TTP, third snake bite and atypical HUS. TPD treatment was applied to all patients. In these four etiological classes, TPD treatment in HELLP syndrome, TTP and snake bite is effective on prognosis and statistically significant improvement in laboratory parameters (p<0.05). In the case of atypical HUS, no statistically significant improvement was observed when the efficacy of TPD on laboratory parameters (p>0.05). When the mortality rates of the four disease groups treated with TPD were examined, it was 9.7% in HELLP Syndrome, 21.7% in TTP, 27.3% in atypical HUS and 0% in snake bite. DISCUSSION Etiologic causes of TMA show regional differences. In our region, TMA group patients are frequently found and although it is espacially seen the most HELLP syndrome in etiology, TTP and snake bite are also common. In patients presenting with thrombocytopenia and MHA, TMA should be suspected and the etiology should be established as soon as possible. However, TPD treatment should be started as soon as possible because of the positive effect on mortality and morbidity without waiting for the definitive TMA etiology. Key words: Thrombotic microangiopathy, Therapeutic plasma exchange, TTP, HELLP syndrome, Snake bite, Atypical Hemolytic Uremic Syndrome