Res Pract Thromb Haemost. 2026 Mar 4;10(2):103406. doi: 10.1016/j.rpth.2026.103406. eCollection 2026 Feb.
ABSTRACT
BACKGROUND: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare, life-threatening thrombotic microangiopathy caused by severe a disintegrin and metalloproteinase with thrombospondin motifs 13 (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: A total of 2771 iTTP episodes were identified. Incidence of iTTP episodes increased from 2.36 to 3.48 per million inhabitants (P = .003), peaking in women aged 30 to 34 and men aged 60 to 64 years. While in-hospital mortality remained stable at 12.8%, complications such as multiorgan failure (33.5%) and need for mechanical ventilation (17.9%) increased significantly. Treatment patterns shifted: the median number of plasma exchange sessions decreased (from 12 to 6), while rituximab use increased (from 24% to 40%, P < .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.
PMID:41940237 | PMC:PMC13049508 | DOI:10.1016/j.rpth.2026.103406