Abstract
Modern therapy for acute TTP has resulted in a dramatic improvement in outcomes, with the combination of plasma exchange, immunosuppression, and caplacizumab being associated with >90% survival rates following an acute episode. TTP is no longer associated with just the acute episode, but requires long-term follow-up. There remains significant morbidity associated with acute TTP, and many patients suffer marked neuropsychological sequelae, including impairment in cognitive functioning, affective disorders, and reduction in health-related quality of life measures. The focus of management beyond the acute phase centres on relapse prevention, via careful monitoring of patients and the use of either ad hoc or regular immunosuppressive therapies. The main therapy used is rituximab, but despite more limited evidence, other immunosuppressive therapies may be required to aim for normalisation of ADAMTS 13 activity. Follow-up with a reduction in ADAMTS 13 activity levels (ADAMTS 13 relapse), rituximab is central to normalisation of activity levels and prevention of a clinical relapse. Fundamental to elective therapy is the role of ADAMTS 13 activity monitoring, and impact of reduced ADAMTS13 activity on end organ damage. This review discusses monitoring and treatment strategy for long-term management of TTP, including the variety of therapies available to maintain remission, prevent relapse and a summary of a long-term treatment pathway.