Continuity of care in immune thrombotic thrombocytopenic purpura ( <scp>iTTP</scp> ): A visual roadmap for survivorship beyond acute management

Immune thrombotic thrombocytopenic purpura (iTTP) is a life-threatening pro-thrombotic disorder due to an autoantibody against the ADAMTS13 protein,1-4 requiring urgent treatment that may include ADAMTS13 replacement (e.g., plasmapheresis by transfusion medicine), immunotherapy, and anti-von willebrand factor therapy in acute hospitalized settings.5 However, there is patient and provider variability and uncertainties in addressing quality of life with survivorship after acute disease, including exacerbations and long-term relapse risk.6-17 The goal of our figure/roadmap is to illustrate iTTP management in several stages, emphasizing care beyond the acute management phase. Long-term complications such as major adverse cardiovascular events, cognitive and neurological impairments (e.g., stroke or dementia), psychiatric sequelae (e.g., depression, anxiety), including healthcare-associated post-traumatic stress disorder and diminished overall quality of life, should be considered in iTTP survivors, along with interval ADAMTS13 activity monitoring and adjustment of immunosuppression as needed. Such challenges may emerge from months to years after the acute episode and may be under-recognized or unrecognized during routine follow-up. Our roadmap highlights the importance of proactive surveillance and supportive care, including neurocognitive screening,15 mental health evaluation,15, 16 and cardiovascular risk assessment,10, 11 tailored to addressing specific patient comorbidities.13 This pathway of the journey is important to highlight as the goal is to prevent any further relapses and avoid any further organ damage. Our roadmap does not directly depict guidelines nor intend to serve as a decision-making tool. Although long-term care strategies for iTTP are still evolving, our roadmap advocates for sustained interdisciplinary collaboration to ensure survivors receive comprehensive, proactive support years after acute disease management.1, 2 Although transfusion medicine may not directly manage long-term survivorship, transfusion medicine specialists remain integral for relapse management and ADAMTS13 testing, working alongside other disciplines to support continuity of care. Minh-Ha Tran has received honoraria from Sanofi. The data that support the findings of this study are available from the corresponding author upon reasonable request.