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Abstract: PO0054

Retrospective Analysis of the Efficiency of Caplacizumab in the Treatment of Acquired Thrombotic Thrombocytopenic Purpura: Results from the REACT-2020 Study Group

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical, Outcomes, and Trials


  • Kuehne, Lucas, University Hospital Cologne, Koeln, Nordrhein-Westfalen, Germany
  • Kaufeld, Jessica Katharina, Hannover Medical School, Medizinische Hochschule Hannover, Hannover, Niedersachsen, Germany
  • Menne, Jan, Hannover Medical School, Medizinische Hochschule Hannover, Hannover, Niedersachsen, Germany
  • Brinkkoetter, Paul T., University Hospital Cologne, Koeln, Nordrhein-Westfalen, Germany
  • Volker, Linus A., University Hospital Cologne, Koeln, Nordrhein-Westfalen, Germany

Group or Team Name

  • REACT-2020 study group

Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare but life-threatening disorder, caused by the formation of inhibitory and occasionally non-inhibitory autoantibodies against ADAMTS13. Despite plasma exchange and immunosuppression, long-term mortality and morbidity associated with acute episodes remain high. Here, caplacizumab – a nanobody approved in Germany in 2018 – has recently expanded the therapeutic options.


Retrospective analysis of the use of caplacizumab in more than 60 patients from more than 30 different medical centers in Germany during acute disease management.


Caplacizumab led to a rapid normalization of the platelet count (median 3, mean 3.78 days). In 34/60 instances, caplacizumab was stopped prior to day 30 with favorable outcome whenever ADAMTS13 activities were >10%. In contrast, 11/34 patients with ADAMTS13 activities <10% at the time of stopping caplacizumab treatment develop a non-favorable outcome comprising disease exacerbation or relapse. In some cases, prolongation of the treatment interval to every other day was feasible and resulted in a sustained reduction of the vWF activity.


Caplacizumab is efficacious in the treatment of aTTP independent of timing and adjunct treatment modalities. Based on this real-world experience and published literature we propose to administer caplacizumab to all patients with an acute episode of aTTP immediately. ADAMTS13 activity measurements are central for a rapid diagnosis in the acute setting but also to tailor disease management. In addition, vWF activity may serve as biomarker for drug monitoring.