Abstract
BACKGROUND: Despite improvement in acute care of immune-mediated thrombotic thrombocytopenic purpura (iTTP), numerous studies showed that patients with iTTP have inferior mental health and health-related quality of life (HRQoL). Caplacizumab led to shorter hospitalization, less plasma exchange, and improved survival in iTTP and might influence long-term HRQoL. OBJECTIVES: We aimed to address the impact of iTTP on HRQoL, posttraumatic stress disease, depression, and anxiety, as well as the possible role of caplacizumab on improving these features. METHODS: We conducted a survey among patients with iTTP enrolled in the French thrombotic microangiopathy registry: patients completed the Short Form (SF)-36 HRQoL, the Hospital Anxiety and Depression Scale screening for anxiety and depression, and the Posttraumatic Stress Disorder Checklist for DSM-IV posttraumatic stress disease questionnaires. Results were compared to those of a sample of the French general population. RESULTS: A total of 101 patients with iTTP in remission (45 patients previously treated with caplacizumab and 56 patients without caplacizumab) and 76 healthy controls were included. Patients with iTTP had significantly lower scores in all domains in the SF-36 survey and higher anxiety and depression scores than healthy controls. Advanced age was associated with improved SF-36 scores, lower anxiety scores and less severe anxiety cases. Furthermore, the use of caplacizumab led to a lower risk of severe anxiety. CONCLUSION: Based on our results, HRQoL is decreased in patients with iTTP, and depression and anxiety are more prevalent. Caplacizumab treatment might influence long-term mental and psychological outcomes in iTTP by shortening the duration of treatment with plasma exchange.