Leuci Alexandre, Millon Antoine, Chopin Alice, Rezigue Hamdi, Alotaibi Ssakher, Dargaud Yesim
UR4609 Hemostasis & Thrombosis Research Unit, Faculté de Médecine Lyon Est, Université Claude Bernard Lyon 1, 69008 Lyon, France.
Service de Chirurgie Vasculaire, Hopital Louis Pradel, Hospices Civils de Lyon, 69500 Lyon, France.
J Clin Med. 2024 Sep 30;13(19):5839. doi: 10.3390/jcm13195839.
Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder of platelet function. The frequent occurrence of alloimmunization due to repeated platelet transfusions is the major complication of the disease. Achieving hemostasis in these patients with anti-GPIIb-IIIa antibodies during surgical procedures is a significant challenge due to the high risk of bleeding. Recombinant activated factor VII (rFVIIa) is an effective agent for achieving hemostasis in alloimmunized Glanzmann's thrombasthenia patients. The key clinical question was to determine whether abdominal aortic aneurysm surgery can be safely performed with rFVIIa in Glanzmann's thrombasthenia patients with anti-GPIIb/IIIa antibodies and whether long-term antiplatelet therapy is suitable for these patients. The patient underwent endovascular aneurysm repair with intensive rFVIIa administration, experiencing neither bleeding nor thrombosis. Data regarding the surgical management of Glanzmann's thrombasthenia patients with anti-GPIIb-IIIa antibodies and the use of antithrombotics in this high-risk population are still very limited. Sharing clinical experience can be valuable for hematologists managing similar cases.
血小板无力症(GT)是一种罕见的常染色体隐性血小板功能障碍疾病。因反复输注血小板导致的同种免疫频繁发生是该疾病的主要并发症。由于出血风险高,在手术过程中,对于这些具有抗血小板糖蛋白IIb-IIIa抗体的患者而言,实现止血是一项重大挑战。重组活化因子VII(rFVIIa)是使同种免疫的血小板无力症患者实现止血的有效药物。关键的临床问题是确定在具有抗血小板糖蛋白IIb/IIIa抗体的血小板无力症患者中,能否使用rFVIIa安全地进行腹主动脉瘤手术,以及长期抗血小板治疗是否适用于这些患者。该患者在强化使用rFVIIa的情况下接受了血管内动脉瘤修复术,未出现出血或血栓形成。关于具有抗血小板糖蛋白IIb-IIIa抗体的血小板无力症患者的手术管理以及在这一高风险人群中使用抗血栓药物的数据仍然非常有限。分享临床经验对于处理类似病例的血液科医生可能很有价值。