Evaluation of clinical, laboratory and treatment results of our patients diagnosed with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare clinical picture classified in the thrombotic microangiopathy (TMA) group, with a very high mortality if not immediately recognized and treated. TTP is clinically caused by severely deficient activity of the ADAMTS-13 protease. It is more common in women and the male/female ratio is 2/3. The most common age of onset is in the 3rd or 4th decade. The classical pentad of TTP, MAHA, is thrombocytopenia, neurologic findings, fever, and renal failure.. The aim of iTTP treatment is to replace the missing ADAMTS13 enzyme in the blood, and to remove or neutralize these antibodies if the etiologic cause is antibodies that block ADAMTS13 activity. PD, corticosteroids, rituximab are used in the treatment and in recent years, caplasizumab has been included in the treatment combination. Immunosuppressive therapies such as cyclophosphamide, vincristine or cyclosporine can be used in patients who are resistant to PD and rituximab. In our study, clinical and laboratory findings and treatment results of 31 patients who were diagnosed with iTTP when they were 18 years of age or older between 1.01.2008 and 1.01. 2023 in Eskişehir Osmangazi University Faculty of Medicine, Department of Internal Medicine, Department of Hematology were evaluated retrospectively. Of 31 patients, 7 (22.6%) were male and 24 (77.4%) were female. The mean age of the patients at the time of diagnosis was 45.13 ±19.07 (16-83) years. There were signs of hemolysis in the peripheral smear of all patients. MAHA and thrombocytopenia were detected in all patients. In the treatment, PD was applied to all patients. The time to start PD after admission to the hospital was 24 (12-24) hours. As a result of the evaluation made between 12 refractory patients (42.9%) and 16 nonrefractory patients (57.7%), a significant difference was found between total bilirubin and direct bilirubin values at the time of diagnosis. As a result of the evaluation between 7 mortal patients (22.6%) and 24 non-mortal patients (77.4%), a difference was found in terms of age at the time of diagnosis. Key Words: Thrombotic thrombocytopenic purpura, therapeutic plasma exchange