Markovitch O, Ellis M, Holzinger M, Goldberger S, Beyth Y
Department of Obstetrics and Gynecology, Meir Hospital, Kfar Saba, Israel.
Am J Hematol. 1998 Mar;57(3):225-7. doi: 10.1002/(sici)1096-8652(199803)57:3<225::aid-ajh8>3.0.co;2-x.
Glanzmann's thrombasthenia is a rare inherited hematological disorder defined by deficiency or abnormality of the glycoprotein (GP) IIb-IIIa complex. Presenting symptoms are hemorrhagic events, mainly epistaxis, purpura, or menorrhagia. We describe the clinical course and management of a 14-year-old girl with Glanzmann's thrombasthenia and severe menorrhagia. Following treatment with 20 U of packed red blood cells, 37 U of platelets, 7 U of fresh frozen plasma, cryoprecipitate, intravenous estrogens, and methylergotrine maleate with no improvement, the uterine cavity was packed for 48 hr. This unusual procedure halted the bleeding and avoided the necessity for a hysterectomy. When treating acute menorrhagia in patients with Glanzmann's thrombasthenia, the physician should be familiar with the characteristics and all treatment modalities for this disorder.
血小板无力症是一种罕见的遗传性血液系统疾病，其特征为糖蛋白（GP）IIb-IIIa复合物缺乏或异常。主要症状为出血事件，主要是鼻出血、紫癜或月经过多。我们描述了一名患有血小板无力症和严重月经过多的14岁女孩的临床病程及治疗情况。在用20单位浓缩红细胞、37单位血小板、7单位新鲜冰冻血浆、冷沉淀、静脉注射雌激素和马来酸甲基麦角新碱治疗后无改善，遂对宫腔进行了48小时填塞。这一不同寻常的操作止住了出血，避免了子宫切除的必要性。在治疗血小板无力症患者的急性月经过多时，医生应熟悉该疾病的特点及所有治疗方式。
