首发症状为鼻出血的血小板无力症3例临床诊治并文献复习

Clinical analysis on Glanzman Thrombasthenia with epistaxis first complaint: three cases report and foreign literature review

目的总结血小板无力症伴鼻出血患儿的临床特征和诊治措施。方法回顾性分析2000~2010年以鼻出血首诊的3例血小板无力症患者临床资料,并复习相关文献,总结其临床特征与诊疗方法。结果例1为双侧弥漫性鼻腔出血且有家族史,例2无明显诱因顽固性鼻出血,例3鼻出血量与创伤程度不相符。表现红细胞及血红蛋白下降,ADP诱导试验血小板聚集率降低或无反应,血小板膜糖蛋白GPⅡb/Ⅲa检测值明显偏低。经鼻腔填塞和输注新鲜血小板后,鼻出血均得到有效控制。结论鼻出血可以是血小板无力症的主要首发症状,实验室检查可以确诊,重组人凝血因子Ⅶa是治疗本病的新方法。

Objective To sum up the clinical features, diagnosis and treatment of Glanzman Thrombasthenia （GT） with epistaxis as the first symptom. Method A retrospective analysis was carried out among 3 children cases of GT with epistaxis as the first symptom, treated in our Hospital from 2000 to 2010, through a summary of their clinical data and with a review on related literatures for such a condition to focus on its diagnosis and treatment. Result Persistent bleeding in both nasal cavities was seen in case 1, with a family ill history of the same condition for this case. Refractory epistaxis was the main complain but without any incentive cause found for case 2. For case 3, the volume of bleeding was massive following once collision to nose by a stuffed toy, though such a degree of trauma being impo^ible to explain her severe nose bleeding. All the 3 cases were seen with erythrocyte number and haemoglobin level declined, and declined reaction, even no reaction, was detected for platelet aggregate rate induced by ADP. Their GPIIb/ 11Ia contents were determined at a lower level. Their epistaxis could be effectively controlled by nose packing and fresh platelet transfusion. Conclusion Epistaxis may be the most commonly seen first symptom among cases with GT. The therapy of recombinant human blood coagulation factor VIIa is a new treating procedure for such a condition.