Korean expert consensus on the management of thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening thrombotic microangiopathy resulting from severe ADAMTS13 deficiency. Although effective treatments, such as therapeutic plasma exchange, immunosuppressive therapy with rituximab, and caplacizumab, have significantly improved survival, important challenges remain. These include limited diagnostic capacity, barriers to the early use of novel therapies, and long-term complications. In Korea, additional difficulties persist due to the restricted availability of ADAMTS13 antibody testing, reimbursement limitations for rituximab, and the absence of caplacizumab in clinical practice. To address these issues, the Korean Society of Hematology Thrombosis and Hemostasis Working Party convened an expert panel to develop consensus recommendations for the diagnosis and management of TTP. The panel reviewed current international guidelines, pivotal clinical studies, and real-world experiences and adapted them to the Korean clinical setting. This consensus statement provides updated recommendations for diagnostic approaches, initial and adjunctive therapies, management of refractory disease, ADAMTS13 monitoring, and long-term follow-up. By integrating international evidence with local circumstances, this document aims to provide Korean clinicians with practical, upto-date guidance to enhance the routine care of patients with TTP.