Kilincaslan Huseyin, Leblebisatan Goksel, Tepeler Abdulkadir, Karakus Suleyman C
Department of Pediatric Surgery, Faculty of Medicine, Bezmialem Vakif University, Istanbul, Turkey.
Blood Coagul Fibrinolysis. 2011 Dec;22(8):735-7. doi: 10.1097/MBC.0b013e32834a5c4c.
Glanzman thrombasthenia is a rare hematologic disorder characterized by qualitative thrombocyte abnormality. Patients present with episodic mucocutaneous bleeding. Thrombosis is a paradox phenomenon observed in patients with Glanzman thrombasthenia and generally considered as a treatment complication. We present a 16-year-old girl referred for severe flank pain beginning after treatment of hematuria due to Glanzman thrombasthenia. The patient underwent endoscopy for further diagnosis and treatment because of the failure of radiologic evaluation. Although the resolution of the large clots was obtained with streptokinase instillation via the ureteral catheter, clot was mobilized with gentle insertion of ureteral catheter in the present case.
Glanzman血小板无力症是一种罕见的血液系统疾病,其特征为血小板质量异常。患者表现为间歇性黏膜皮肤出血。血栓形成是Glanzman血小板无力症患者中观察到的一种矛盾现象,通常被认为是一种治疗并发症。我们报告一名16岁女孩,因Glanzman血小板无力症导致血尿,在治疗后开始出现严重的侧腹疼痛。由于影像学评估失败,患者接受了内镜检查以进行进一步诊断和治疗。尽管通过输尿管导管注入链激酶使大血块溶解,但在本病例中,通过轻柔插入输尿管导管使血块移动。