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围手术期使用重组活化因子 VII 治疗伴血小板抵抗的 Glanzmann 血小板无力症患者:病例报告。

Perioperative administration of recombinant activated factor VII in a Glanzmann's thrombasthenia patient with platelet refractoriness: case report.

机构信息

CET/SBA Integrado de Uberaba, Uberaba, MG, Brazil; Universidade Federal do Triângulo Mineiro (UFTM), Disciplina de Anestesiologia, Uberaba, MG, Brazil.

CET/SBA Integrado de Uberaba, Uberaba, MG, Brazil; Hospital Regional Jose Alencar, Uberaba, MG, Brazil.

出版信息

Braz J Anesthesiol. 2022 Nov-Dec;72(6):823-825. doi: 10.1016/j.bjane.2021.09.021. Epub 2021 Nov 28.

Abstract

Glanzmann's Trombasthenia (GT) is a genetic disorder, that develops with a tendency toward bleeding and is characterized by the absence or decrease in platelet aggregation. Surgical bleeding may be difficult to control. Platelet transfusion is the main treatment, albeit refractoriness can occur. We describe the case of a patient with GT and platelet refractoriness, who was submitted to radical prostatectomy and dental extraction. The perioperative treatment with apheresis platelet concentrate and activated recombinant factor seven allowed the procedures to be performed uneventfully. We discuss the complexity of the case and the treatment option.

摘要

格拉赞曼氏血小板无力症(GT)是一种遗传性疾病,表现为出血倾向,其特征为血小板聚集缺失或减少。外科手术出血可能难以控制。血小板输注是主要治疗方法,但可能会出现抵抗。我们描述了一名 GT 伴血小板抵抗患者的病例,该患者接受了根治性前列腺切除术和拔牙。采用血小板单采浓缩物和重组活化因子 VII 的围手术期治疗使手术顺利进行。我们讨论了该病例的复杂性和治疗选择。

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