Department of Nephrology, Rajagiri Hospital, Aluva, Cochin, Kerala, India.
Saudi J Kidney Dis Transpl. 2020 Mar-Apr;31(2):545-548. doi: 10.4103/1319-2442.284035.
Coagulopathy in the nephrotic syndrome (NS) is very rare. Simultaneous prolongation of both prothrombin time and activated partial thromboplastin time suggests common coagulation pathway abnormality such as liver dysfunction, Vitamin K deficiency, disseminated intravascular coagulation, or primary fibrinolysis. This results in difficulty in proceeding with renal biopsy and tissue diagnosis. We report one such case of NS with coagulopathy and refractoriness to correction with blood products, which led us to make a diagnosis of AL amyloidosis, which was confirmed with abdominal fat pad biopsy and other work-up.
肾病综合征(NS)中的凝血异常非常罕见。同时延长凝血酶原时间和活化部分凝血活酶时间提示常见的凝血途径异常,如肝功能障碍、维生素 K 缺乏、弥漫性血管内凝血或原发性纤溶。这导致进行肾活检和组织诊断变得困难。我们报告了一例 NS 合并凝血异常和对血液制品纠正无反应的病例,这导致我们做出了 AL 淀粉样变性的诊断,腹部脂肪垫活检和其他检查证实了这一诊断。