2025 focused update of the 2020 ISTH guidelines for management of thrombotic thrombocytopenic purpura

The International Society on Thrombosis and Haemostasias (ISTH) has published updated guidelines for the management of thrombotic thrombocytopenic purpura (TTP). The updates to this guideline, originally published in 2020, aim to standardize the therapeutic approach at an international level. In turn, this will increase access to care, reduce hospitalizations and ultimately lower mortality rates for patients with TTP.

The updates to these guidelines focus on data from recent trials showing the use of ADAMTS13 in prophylaxis of congenital TTP (cTTP). As a result, the guidelines now favor the prophylactic use of fresh frozen plasma over a watch-and-wait strategy in patients with cTTP during remission. These changes will lower the risk of acute episodes and reduce the potential long-term complications associated with cTTP. Additionally, shared decision-making should include the benefits, potential harm and the burden of care.