Objective To investigate the effectiveness and safety of plasma exchange for thrombotic microangiopathy and anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis,which require renal replacement therapy.Methods This is a retrospective study.The clinical data of four patients who underwent plasma exchange at the Blood Purification Center,North Branch of the First Affiliated Hospital of Anhui Medical University,from June 2021 to February 2022 were collected and analyzed.The records included a total of 19 treatments performed,including 15 treatments with a single plasma exchange regimen used in 3 patients and 4 treatments with double filtration plasmapheresis used in 1 patient.Changes in condition before and after treatment,as well as corresponding biochemical test results,were recorded.Results In four patients,clinical symptoms and biochemical indicators improved.Three patients required maintenance hemodialysis,while one patient showed remarkable clinical efficacy.After treatment,hemoglobin level increased from 76 g/L to 83 g/L,blood creatinine level decreased from 703.6 μmol/L to 526.2 μmol/L,anti-SM antibody decreased from>400 RU/mL to<2 RU/mL,anti-myeloperoxidase antibody decreased from 255.49 RU/mL to 15.64 RU/mL,and perinuclear anti-neutrophil cytoplasmic antibody turned negative.During treatment,there were two cases of allergic reactions,which were signifiicantly alleviated after treatment.Conclusion Plasma exchange is safe and effective against thrombotic microangiopathy and anti-neutrophil cytoplasmic antibody-associated vasculitis,which require renal replacement therapy.