Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder of red cell damage (haemolysis), low platelets (with risk risk of bleeding) and thrombosis in small blood vessels causing kidney and nervous system and other vital organs. Urgent and repeated large-volume plasma exchange (PE) is the critical component of current treatment. Anecdotally, PE-related adverse events, including allergic reactions, transfusion-related acute lung injury, citrate anticoagulant effects, haemodynamic instability, and procedural problems are common, and they are almost certainly underreported in Australia. The effects of different plasma and non-plasma replacement fluids, volumes of exchange, and types of venous access have not been studied in the Australian setting. The aim of this project is to describe and determine the incidence of PE-associated adverse events in Australian patients being treated for TTP. In addition, it will evaluate any association between difference in PE practice (e.g. use of premedication, choice of replacement fluid, type of venous access) and incidence of adverse events during PE. Finally, it aims to describe and determine the impact of adverse events on patient-reported quality of life (QoL). The rationale for this project is that PE-related adverse events are not uncommon, and may be serious, yet optimal practice for PE remains to be determined. Furthermore, information from the TTP/Thrombotic Microangiopathy (TMA) Registry shows substantial variation in clinical practice between individual clinicians and institutions across Australia. Results will inform management of TTP, other TMAs, and other conditions for which PE is performed, and contribute to national haemovigilance data and development of clinical practice guidelines.
haematology transfusion TTP
School of Public Health and Preventive Medicine » Epidemiology and Preventive Medicine
The Alfred Centre, Prahran