Epidemiology, therapy, and outcome of immune-mediated thrombotic thrombocytopenic purpura at population level in Germany

Background: (ADAMTS-13) deficiency due to autoantibodies. Although therapeutic advances have improved outcomes, data on population-level trends in epidemiology, therapy, and outcomes remain limited. Objectives: We assessed temporal trends in incidence, treatment strategies, and acute in-hospital outcomes of iTTP episodes at the population level in Germany from 2011 to 2021. Methods: This retrospective population-based cohort study used nationwide inpatient data from the German Federal Statistical Office. iTTP cases were identified by International Classification of Disease, Tenth Revision, coding combined with documentation of plasma exchange procedures. Trends were analyzed using linear regression. Results: < .001). Caplacizumab was used in 34.7% of patients in 2020 to 2021. Male patients were older and had more severe courses. Outcomes were comparable between university and nonuniversity hospitals. Conclusion: The incidence and severity of iTTP episodes have increased in Germany over the past decade, and treatment strategies have evolved. These findings warrant further research to optimize acute management and understand long-term outcomes.