Javed Asim, Ayyub Muhammad, Abrar S, Mansoor M, Khan Badshah, Hussain T
Combined Military Hospital, Rawalpindi, Pakistan.
J Ayub Med Coll Abbottabad. 2009 Apr-Jun;21(2):171-3.
Glanzmann's thrombasthenia is an autosomal recessive inherited platelet function defect. Though, quantitatively normal, the aggregation ability of platelets is reduced leading to bleeding episodes requiring transfusion of platelet concentrates. We describe a case of 13-year-old girl who had recurrent episodes of epistaxis since birth and was managed with multiple platelet concentrate transfusions and recently admitted with severe epistaxis refractory to platelet transfusion. At this stage administration of recombinant activated factor VII (fVIIa) was considered, which was initially given at 90 microg/kg dose with little control of bleeding but subsequent second dose of 120 microg/kg was administered with excellent response and immediate control of bleeding.
血小板无力症是一种常染色体隐性遗传性血小板功能缺陷病。虽然血小板数量正常，但其聚集能力下降，导致出血发作，需要输注血小板浓缩物。我们描述了一名13岁女孩的病例，她自出生以来反复出现鼻出血，接受了多次血小板浓缩物输血治疗，最近因严重鼻出血入院，对血小板输血治疗无效。在此阶段，考虑给予重组活化因子VII（fVIIa），最初以90微克/千克的剂量给药，对出血控制不佳，但随后给予120微克/千克的第二剂，反应良好，出血立即得到控制。
