Fatal cerebral hemorrhage in a patient with thrombotic thrombocytopenic purpura with a normal platelet count during treatment with caplacizumab

Abstract

Acquired thrombotic thrombocytopenic purpura (aTTP) is a thrombotic microangiopathy with a severe mortality and morbidity. Caplacizumab has recently been approved in the Netherlands as a new therapeutic option in patients with life-threatening organ failure due to aTTP. We describe the case of a 50 year old patient with aTTP who was referred to our hospital for treatment with caplacizumab. After undergoing treatment with plasmapheresis, prednisolone, rituximab and caplacizumab, her platelet count recovered and she was ready to be discharged. Unfortunately, before discharge she developed a fatal intra-cerebral hemorrhage. Fatal hemorrhage as an adverse event of caplacizumab has not been described before. Up to now there is no evidence-based treatment for caplacizumab induced heavy bleeding.

Keywords:

• Adverse drug event
• bleeding
• caplacizumab
• cerebral hemorrhage
• thrombotic thrombocytopenic purpura

Disclosure of interest

The authors report no conflict of interest.

Author contribution

Kim Ditzel wrote the manuscript. Dirk Jan Mons contributed to writing the manuscript and reviewed the manuscript. Rob Fijnheer reviewed the manuscript.