Glanzmann’s Thrombocytopenia: A Case Report

Preeti Tiwari, Manav Singh, S. Nayak, G. S. Chakraborthy
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Abstract

Glanzmann’s thrombocytopenia is a platelet disorder in which platelets have qualitative or quantitative deficiencies of the fibrinogen receptor α2bβ3. Most cases are hereditary but can be acquired also. This disorder in named after Dr.Eduard Glanzmann, who first described it in 1918. Its global prevalence is estimated to be one in a million with higher prevalence rate in population where consanguinity marriage is common. It has good prognosis with proper supportive care. Case Summary: A 17-year male patient had come with complaint of severe epistaxis randomly for hours in a known case of Glanzmann’s thrombocytopenia which was diagnosed 10 years ago. With epistaxis he also had complaints of weakness in upper limbs and gastrocnemius muscle Diagnosis: Glanzmann’s thrombocytopenia Treatment: Inj. Etamsylate 500mg BD, Inj. Hemocoagulase 1IU OD, Inj. Tranexamic acid 500mg OD, Tab. Vitamin C BD, Tab. Calcium gluconate 500mg BD, Syp. Vitamin D3 and was transfused with PCV (A+) total 12 pint (200ml) and platelets 2 pint (200ml) Keywords: Glanzmann’s thrombocytopenia, Epistaxis, Tranexamic acid, Congenital, Platelet Disorder.
格兰兹曼血小板减少症:病例报告
格兰兹曼血小板减少症是一种血小板疾病,血小板中的纤维蛋白原受体α2bβ3存在定性或定量缺陷。大多数病例是遗传性的,但也可能是获得性的。这种疾病以爱德华-格兰兹曼博士的名字命名,他于 1918 年首次描述了这种疾病。据估计,其全球发病率为百万分之一,在近亲结婚常见的人群中发病率更高。通过适当的支持性治疗,预后良好。病例摘要:一名 17 岁的男性患者主诉说,他在 10 年前被诊断出患有格兰兹曼血小板减少症,并随机出现严重鼻衄,持续数小时。在鼻衄的同时,他还主诉上肢无力和腓肠肌诊断:格兰兹曼血小板减少症 治疗:注射依他霉素 500 毫克(BD),注射用血凝酶 1I血液凝固酶 1IU OD,氨甲环酸注射液 500 毫克 BD。氨甲环酸 500 毫克口服液,维生素 C BD 片剂,维生素 C 片剂。维生素 C BD、片剂葡萄糖酸钙 500 毫克 BD,维生素 D3。维生素 D3,并输注了 PCV(A+)共 12 品脱(200 毫升)和血小板 2 品脱(200 毫升):格兰兹曼血小板减少症 鼻衄 氨甲环酸 先天性 血小板异常
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