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患有Glanzmann血小板无力症和抗糖蛋白IIb/IIIa抗体患者的肾盂切开取石术:重组活化因子VII和血小板输注的最短治疗时长

Pyelolithotomy in a patient with Glanzmann thrombasthenia and antiglycoprotein IIb/IIIa antibodies: the shortest possible duration of treatment with recombinant activated factor VII and platelet transfusions.

作者信息

Devecioğlu Omer, Unüvar Ayşegül, Anak Sema, Bilge Ilmay, Ander Haluk, Ziylan Orhan

机构信息

Division of Hematology/Oncology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey.

出版信息

Turk J Pediatr. 2003 Jan-Mar;45(1):64-6.

Abstract

Transfusion of platelet concentrates remains the first-line therapy for Glanzmann thrombasthenia in case of bleeding or preparation for surgery. However, development of antibodies to platelet glycoprotein (Gp) IIb/IIIa complex or human leukocyte antigens (HLA) is frequent and the main cause of platelet refractoriness. Recombinant activated factor VII (rFVIIa) is a potent alternative for patients with Glanzmann thrombasthenia with anti-platelet antibodies. We describe a case of Glanzmann thrombasthenia with alloantibodies to platelet Gp IIb/IIIa complex who underwent a successful pyelolithotomy operation under the coverage of recombinant activated factor VIIa and platelet transfusions.

摘要

对于患有Glanzmann血小板无力症且有出血情况或准备进行手术的患者,输注血小板浓缩物仍然是一线治疗方法。然而,血小板糖蛋白(Gp)IIb/IIIa复合物或人类白细胞抗原(HLA)抗体的产生很常见,并且是血小板输注无效的主要原因。重组活化因子VII(rFVIIa)是患有抗血小板抗体的Glanzmann血小板无力症患者的有效替代治疗方法。我们描述了一例患有血小板Gp IIb/IIIa复合物同种抗体的Glanzmann血小板无力症患者,该患者在重组活化因子VIIa和血小板输注的保障下成功进行了肾盂切开取石术。

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