Devecioğlu Omer, Unüvar Ayşegül, Anak Sema, Bilge Ilmay, Ander Haluk, Ziylan Orhan
Division of Hematology/Oncology, Istanbul University Istanbul Faculty of Medicine, Istanbul, Turkey.
Turk J Pediatr. 2003 Jan-Mar;45(1):64-6.
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.
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。