Plasma exchange/infusion Although plasma exchange/infusion (PE/PI) is frequently used, there are no controlled trials of its safety or efficacy in<unk> . Even though PE/PI often partially controls some of the hematological manifestations of<unk>  in some patients, its effectiveness has not been demonstrated in terms of inducing total disease remission. PE/PI is associated with significant safety risks, including risk of infection, allergic reactions, thrombosis, loss of vascular access, and poor quality of life. Importantly, terminal complement activation has been shown to be chronically present on the surface of platelets in patients with<unk>  who appear to be clinically well while receiving chronic PE/PI. Guidelines issued by the European Paediatric Study Group for HUS recommend rapid administration of plasma exchange or plasma infusion (PE/PI), intensively administered daily for 5 days and then with reducing frequency. However, the American Society for Apheresis offers a "weak" recommendation for plasma exchange to treat<unk> , due to the "low" or "very low" quality of evidence supporting its use. Although some patients experienced improvements in red blood cell and platelet counts, plasma therapies generally did not result in full remission. Monoclonal antibody therapy Eculizumab (Soliris) appears to be useful for<unk>  <unk> . In September 2011 the U.S. Food and Drug Administration (FDA) approved it as an orphan drug to treat people with<unk> . This approval was based on two small prospective trials of 17 people and 20 people. In the UK, NICE issued guidance on the use of Eculizumab for treating<unk> , based on five evidence sources, including those used by the FDA No randomised controlled trials were identified. All prospective studies were phase 2, open‑label, non‑randomised, single‑arm studies that included patients with different clinical baseline characteristics. The prospective studies lasted 26 weeks; however, patients were allowed to continue treatment with eculizumab in a long‑term extension study. Ravulizumab-cwvz (Ultomiris) is a second generation monoclonal antibody for<unk>  made by Alexion pharmaceuticals, Inc. The target of ravulizumab-cwvz is the same eculizumab (Soliris) with changes to the structure of the antibody resulting in a longer serum half life and therefore reduced dosing regimen. Dialysis Patients with<unk>  who have ESRD are generally consigned to lifelong dialysis, which carries a 5-year survival rate of 34–38%, with infections accounting for 14% of deaths. These patients also remain at ongoing risk of non-kidney systemic complications of the disease. Kidney transplantation Despite its history of use in patients with<unk> , kidney transplantation does not address the continued and uncontrolled complement activation that leads to progressive, systemic TMA. As many as 90% of patients with<unk>  and who are not treated with Soliris or Ultomiris, experience TMA in the transplanted organ, leading to transplant failure. Patients who have undergone kidney transplantation are still at continued risk of neurological, gastrointestinal, and cardiovascular complications and, importantly, premature mortality. Following kidney transplantation, the ongoing, uncontrolled, chronic complement activation associated with<unk>  causes graft loss in 66% of children and 55% of adults, as well as continued inflammatory and TMA insult to other organs. Combined liver-kidney transplantation is only available to very few patients, due to the limited supply of solid organs. In addition, there is a substantial near-term risk of mortality, which many physicians and patients consider excessive. In recent years, some transplant centers have begun to administer eculizumab to patients with TMA who receive a kidney transplant. This strategy has been effective in preventing TMA recurrences in these patients. Terminal complement inhibition Patients using either eculizumab or ravulizumab for the treatment of<unk>  showed improvements in kidney function even avoiding dialysis and minimizing death. Markers of disease activity in the blood also had a great improvement. However, the only available evidence has substantial bias and low quality and therefore there should be careful considerations for futures studies in treatment duration, adverse outcomes and risk of disease recurrence associated with this treatment.
Atypical hemolytic uremic syndrome
Thrombotic thrombocytopenic purpura
Hemolytic–uremic syndrome
Post-transfusion purpura
Severe fever with thrombocytopenia syndrome
Cold autoimmune hemolytic anemia
Transfusion-dependent anemia
Immune reconstitution inflammatory syndrome