血小板无力症致月经过多临床诊治研究  

A study on diagnosis and treatment of menorrhagia caused by glanzmann thrombasthenia

作　　者：王德朋[1] 杨春霞[2] 田秦杰[2] 

机构地区：[1]山东省淄博市妇幼保健院,山东淄博255029 [2]北京协和医院,北京100730

出　　处：《中国妇幼健康研究》2008年第4期376-378,共3页Chinese Journal of Woman and Child Health Research

摘　　要：血小板无力症是一种常染色体隐性遗传疾病,是由于先天性血小板膜糖蛋白GPⅡb/Ⅲa(简称GPⅡb/Ⅲa)基因缺陷,使得GPⅡb/Ⅲa异常,引起血小板聚集功能异常而导致的出血性疾病。该病好发于青少年,临床表现主要为自幼皮肤、粘膜反复出血,在妇产科就诊的患者多以月经过多为主诉,明确诊断主要依靠实验室检查,治疗以输血和(或)输血小板对症支持治疗为主,妇科处理常以雌、孕激素止血、曼月乐环和手术切除子宫为主。为提高妇产科医师对血小板无力症的诊治认识,该文就此疾病作以综述。Glanzmann thrombasthenia（GT） is an autosomal recessive hereditary hemorrhagic disorder characterized by severe reduction or absence of platelet aggregation in response to multiple physiologic agonists due to qualitative or quantitative abnormalities of platelet membrane glycoprotein （GPⅡb/Ⅲa ）, presenting symptoms are hemorrhagic events. This disease is prone to be seen in adolescents and its clinical manifestations are mainly recurrent dermorrhagia and mucohemorrhagia from early stage of life of the patients and the chief complaints of the patient who visited gynecologists or obstetricians was mainly menorrhagia. Determinedl diagnosis depends upon laboratory examination and the treatment of the disease is mainly supporting therapy and symptomatic therapy such as blood transfusion and （or） platelet transfusion. Gynecological management includes estrogen-progesterone hemostasis, Lng-IUS and hysterectomy. In order to raise recognition of gynecologists and obstetricians for diagnosis and treatment of GT, this article reviewed diagnosis and treatment of the disease.

关 键 词：血小板无力症 血小板膜糖蛋白GPⅡb/Ⅲa 血小板聚集 月经过多 

分 类 号：R558[医药卫生—血液循环系统疾病] R711.51[医药卫生—内科学]