Evaluation of therapeutic plasmaferuses in thrombotic thrombocytopenic purpura

Objective: Thrombotic thrombocytopenic purpura (TTP) is a rare and fatal disease with microangiopathic hemolytic anemia, thrombocytopenia, fever, neurological findings and renal failure. Therapeutic plasma exchange (plasmapheresis) in TTP therapy is accepted as a standard treatment method. In this study, the effect of plasmapheresis on clinical and biochemical parameters and its results were evaluated. Materials and Methods: Patients diagnosed with TTP between 2009-2017 were enrolled in Çukurova University Faculty of Medicine internal medicine hematology department, internal medicine intensive care unit and other service and intensive care units. Before and after each plasmapheresis procedure; peripheral blood smear, whole blood count, BUN, creatinine, LDH, AST, ALT levels were evaluated. Patients were evaluated retrospectively in terms of gender, age, diagnosis and treatment history, frequency and number of apheresis application, treatment response, complications during the procedure. Results: A total of 47 patients were included in this study. The M / F ratio of the patients was 23/24 (48.9% / 51.1%) and the mean age was 39 ± 14.5. The response status was defined as refracture in 14 patients (29.8%), relapse in 2 patients (4.3%), exacerbation in 12 patients (25.5%) and remission in 19 patients (40.4%). 14 patients has been died and the mortality rate was higher than the literature (10%). 4.3% of the patients were relapsed and were found to be lower than the literature. The patients' remission rate (40.4%) was lower than similar literature (80%). Steroid therapy showed positive results in response, whereas immunosuppressive treatment decreased relapsed responses and these results were similar to the literature. Conclusion: Therapeutic apheresis is the standard treatment for TTP disease. The mortality rate in TTP patients has decreased with therapeutic plasmapheresis treatment. Successful results are obtained with steroid treatment and immunosuppressive treatment in cases resistant to therapeutic plasmapheresis treatment. The positive results obtained with the combination of therapeutic plasmapheresis, steroids and immunosuppressive therapy in this study are statistically significant. Key words: Thrombotic Thrombocytopenic Purpura, Therapeutic Plasma, Exchange, steroid, immünosuppressive treatment, rituximab