Boberg Erik, Kimiaei Adrian, Karlström Cecilia, Ljungqvist Maria, Ågren Anna, Bruzelius Maria
Department of Haematology Karolinska University Hospital Stockholm Sweden.
Division of Clinical Immunology, Department of Laboratory Medicine Karolinska Institutet Stockholm Sweden.
EJHaem. 2024 Jun 10;5(4):768-771. doi: 10.1002/jha2.949. eCollection 2024 Aug.
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.
卡泊珠单抗可减少血栓性血小板减少性紫癜（TTP）治疗期间治疗性血浆置换（TPE）的需求，所需TPE次数较少，并缩短住院时间。因此，它被推荐作为标准治疗的一部分。然而，其对止血的治疗效果可能会使初始治疗复杂化。我们报告一例免疫介导的TTP女性患者，在更换中心静脉导管后接受卡泊珠单抗治疗时发生了胸腔内出血。使用血管性血友病因子（vWF）浓缩物逆转了血管性血友病因子（vWF）：糖蛋白Ib突变体（GPIbM）活性降低，出血停止。不幸的是，vWF替代联合停用卡泊珠单抗可能导致随后广泛的静脉血栓栓塞。在卡泊珠单抗治疗期间，针对出血和血栓形成的风险降低策略至关重要，而紧急vWF替代会增加与TPE相关的本就很高的血栓形成风险。
