Duman Erdem Nail, Saylan Sedat, Cekic Bahanur
Faculty of Medicine, Department of Anesthesiology, Karadeniz Technical University, Trabzon, Turkey.
Rev Bras Anestesiol. 2012 Jul;62(4):548-53. doi: 10.1016/S0034-7094(12)70154-X.
Glanzmann's thrombasthenia (GT) is an autosomal recessively inherited platelet disorder. There is not any specific treatment. Platelet transfusion is currently the standard treatment when bleeding does not respond to local measures and/or antifibrinolytic treatment, although it may result in alloimmunization. Recombinant activated factor VII (rFVIIa) might be used to avoid recurrent platelet transfusion.
We present early treatment with low-dose rFVIIa additional to platelet transfusion in a 5-year-old pediatric case with diagnosis of GT who developed prolonged bleeding under an elective adenoidectomy surgery. A total dose of 1,200μg (60μg.kg(-1)) rFVIIa could successfully stop bleeding, what can be accepted as low dose usage.
Such case reports may encourage the use of early treatment with low doses of rFVIIa in severe bleeds that did not stop despite of platelet transfusion, as well as in preventing bleeding in surgical procedures in patients with GT. Actually, additional studies are needed to define the minimal effective dose and attempts to determine the lowest effective dose may be encouraged by the result of this case, considering financial restrictions in the health care system.
Glanzmann血小板无力症（GT）是一种常染色体隐性遗传的血小板疾病。目前尚无特效治疗方法。当出血对局部措施和/或抗纤溶治疗无效时，血小板输注是当前的标准治疗方法，尽管这可能会导致同种免疫。重组活化因子VII（rFVIIa）可用于避免反复进行血小板输注。
我们报告了1例5岁诊断为GT的儿科患者，在择期腺样体切除手术中出现长时间出血，除血小板输注外，早期使用低剂量rFVIIa进行治疗。1200μg（60μg·kg⁻¹）的rFVIIa总剂量成功止血，可视为低剂量使用。
此类病例报告可能会促使人们在血小板输注后仍无法止血的严重出血情况以及GT患者的外科手术中预防出血时，使用低剂量rFVIIa进行早期治疗。实际上，鉴于医疗保健系统的经济限制，需要进一步研究来确定最小有效剂量，而该病例的结果可能会鼓励尝试确定最低有效剂量。
