Division of Hematology, Department of Medicine, Mayo Clinic Rochester, Rochester, Minnesota, USA.
Department of Anesthesiology and Perioperative Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Transfusion. 2023 Sep;63(9):1773-1777. doi: 10.1111/trf.17477. Epub 2023 Jul 10.
AL amyloidosis is associated with acquired factor X (FX) deficiency. Experience related to its management is limited to case reports and series using prothrombin complex concentrate, fresh frozen plasma, plasma exchange, recombinant activated factor seven, and desmopressin with limited and variable efficacy. FX concentrate has not been widely used in its management.
We report our experience with the perioperative use of FX concentrate (Coagadex) in two patients with AL amyloidosis-associated acquired FX deficiency requiring surgery, using their individual pharmacokinetic studies to manage perioperative hemostasis. Pharmacokinetic studies involved obtaining post-infusion FX activity at 10 min, 2, and 4 h following the administration of FX concentrate to calculate the FX half-life.
Both patients' plasma FX activity was successfully increased to provide perioperative hemostatic support. Monitoring FX activity post-surgery was also utilized to maintain FX activity levels to prevent post-operative bleeding.
Pharmacokinetic studies have a useful role in tailoring preoperative FX repletion in patients with AL amyloidosis associated with acquired FX deficiency.
AL 淀粉样变性可导致获得性因子 X（FX）缺乏。目前针对其管理的经验仅限于使用凝血酶原复合物浓缩物、新鲜冷冻血浆、血浆置换、重组激活因子 VII 和去氨加压素的病例报告和系列研究，这些方法的疗效有限且各不相同。FX 浓缩物尚未广泛用于其管理。
我们报告了在两名需要手术的 AL 淀粉样变性相关获得性 FX 缺乏症患者中，围手术期使用 FX 浓缩物（Coagadex）的经验，使用他们各自的药代动力学研究来管理围手术期止血。药代动力学研究包括在给予 FX 浓缩物后 10 分钟、2 小时和 4 小时时获得输注后 FX 活性，以计算 FX 半衰期。
两名患者的血浆 FX 活性均成功升高，以提供围手术期止血支持。术后还监测 FX 活性以维持 FX 活性水平，以预防术后出血。
药代动力学研究在为与获得性 FX 缺乏相关的 AL 淀粉样变性患者进行术前 FX 补充方面具有重要作用。
