Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by microangiopathic hemolytic anemia and severe thrombocytopenia caused by microthrombus formation. It can be congenitalas a result of a mutation in the ADAMTS13 protein (A disintegrant and metalloprotease with Thrombo Spondin type 1), cleaving von Willebrand factor (VWF) multimers, or acquired by the development of autoantibodies directed against ADAMTS13. The original description of TTP in 1924 by Moschowitz describes in detail the pentad of signs and symptoms, which include anemia, thrombocytopenia, fever, neurological disorder, and kidney damage. The diagnosis of TTP should be suspected in the presence of the clinical features described above. Due to clinical urgency, treatment should be initiated empirically before the diagnosis is confirmed by laboratory examination. Insufficient ADAMTS13 activity of <10% is required to diagnose TTP. Further investigation of anti-ADAMTS13 IgG inhibitory autoantibodies is needed to document the mechanism of ADAMTS13 deficiency. The gold standard in the treatment of TTP is the therapeutic exchange of plasma, or plasmapheresis. It is an invasive therapeutic method that separates plasma from blood cells. Pathogenic antibodies or other large molecules are removed and plasma is replaced by human albumin and / or fresh frozen plasma. The nurse has an extremely important role in the treatment of patients with thrombotic thrombocytopenic purpura, especially in the treatment using plasmapheresis. She assists the physician during the placement of a dialysis central venous catheter, is responsible for providing plasma or albumin from the transfusion service, and preparing the device and extracorporeal circuit for plasmapheresis. Also, nurse interventions include assessing and preparing patients for plasmapheresis and monitoring for complications.