Iatrogenic hemorrhage and extensive venous thromboembolism during iTTP treatment with caplacizumab—A case report

• Published in: EJHaem Vol. 5; no. 4; pp. 768 - 771
• Main Authors: Boberg, Erik, Kimiaei, Adrian, Karlström, Cecilia, Ljungqvist, Maria, Ågren, Anna, Bruzelius, Maria
• Format: Journal Article
• Language: English
• Published: United States John Wiley & Sons, Inc 01.08.2024; Wiley
• Subjects: adverse drug event; Anticoagulants; Bleeding; Blood platelets; Catheters; Clinical trials; Disease prevention; Drug dosages; Embolisms; Emergency medical care; Glycoproteins; Hemoglobin; Hemorrhage; Hemostasis; Medicin och hälsovetenskap; Molecular weight; Patients; Plasma; Thrombocytopenic purpura; Thromboembolism; Thrombosis; Thrombotic thrombocytopenic purpura; Ultrasonic imaging; Veins & arteries; venous thromboembolism; Von Willebrand factor; adverse drug event; thrombotic thrombocytopenic purpura; venous thromboembolism; von Willebrand factor; hemorrhage
• ISSN: 2688-6146; 2688-6146
• DOI: 10.1002/jha2.949

Caplacizumab reduces the need for therapeutic plasma exchange (TPE) during treatment for thrombotic thrombocytopenic purpura (TTP), associates with fewer required TPE, and shortens hospital stay. It is therefore recommended as part of standard care. However, the treatment effects on hemostasis may complicate initial management. We present a case of a woman with immune‐mediated TTP who developed an intrathoracic hemorrhage on caplacizumab treatment after replacement of her central venous catheter. Reduced von Willebrand factor (vWF):glycoprotein Ib mutant (GPIbM) activity was reversed using vWF concentrate and the bleeding stopped. Unfortunately, vWF substitution in combination with caplacizumab discontinuation likely contributed to subsequent extensive venous thromboembolism. Risk‐reducing strategies against both bleeding and thrombosis are crucial during caplacizumab treatment, and emergency vWF substitution increases the already high risk of thrombosis associated with TPE.