Swathi Jami, Gowrishankar A, Jayakumar S A, Jain Karun
Department of Internal Medicine, Stanley Medical College, Chennai, Tamil Nadu, India.
Department of Orthopaedics, JSS Medical College, Mysore, Karnataka, India.
Ann Afr Med. 2017 Oct-Dec;16(4):196-198. doi: 10.4103/aam.aam_59_16.
Glanzmann's thrombasthenia (GT) is a rare bleeding disorder, which is characterized by a lack of platelet aggregation. It is characterized by qualitative or quantitative abnormalities of the platelet membrane glycoprotein IIb/IIIa. Physiologically, this platelet receptor normally binds several adhesive plasma proteins, and this facilitates attachment and aggregation of platelets to ensure thrombus formation at sites of vascular injury. The lack of resultant platelet aggregation in GT leads to mucocutaneous bleeding whose manifestation may be clinically variable, ranging from easy bruising to severe and potentially life-threatening hemorrhages.
To highlight this rare but potentially life-threating disorder, GT.
We report a case of GT that was first detected because of the multiple episodes of gum bleeding. The patient was an 18-year-old girl who presented with a history of repeated episodes of gum bleeding since childhood. Till the first visit to our hospital, she had not been diagnosed with GT despite a history of bleeding tendency, notably purpura in areas of easy bruising, gum bleeding, and prolonged bleeding time after abrasions and insect stings. GT was diagnosed on the basis of prolonged bleeding time, lack of platelet aggregation with adenosine di phosphate, epinephrine and collagen.
GT should always be considered as differential diagnosis while evaluating any case of bleeding disorder.
Glanzmann血小板无力症(GT)是一种罕见的出血性疾病,其特征是血小板聚集功能缺乏。它的特点是血小板膜糖蛋白IIb/IIIa存在定性或定量异常。在生理情况下,这种血小板受体通常会结合几种黏附性血浆蛋白,这有助于血小板的附着和聚集,以确保在血管损伤部位形成血栓。GT中血小板聚集功能的缺乏会导致皮肤黏膜出血,其表现临床上可能各不相同,从容易出现瘀斑到严重的、可能危及生命的出血。
强调这种罕见但可能危及生命的疾病——GT。
我们报告一例因多次牙龈出血首次被发现的GT病例。患者是一名18岁女孩自童年起就有反复牙龈出血史。直到首次到我院就诊时,尽管有出血倾向病史,特别是易出现瘀斑部位的紫癜、牙龈出血以及擦伤和虫咬后出血时间延长,但她仍未被诊断为GT。根据出血时间延长、血小板对二磷酸腺苷、肾上腺素和胶原缺乏聚集功能,诊断为GT。
在评估任何出血性疾病病例时,应始终将GT作为鉴别诊断考虑。