PDF(Pubmed)
Abstract：
Amyloidosis with renal involvement is a well-known cause of nephrotic syndrome. Immunoglobulin light-chain amyloidosis (AL), which is a result of monoclonal light-chain deposition in the kidney from plasma cell dyscrasia, is rare before the age of 40 and typically occurs in old patients. Most cases of renal amyloidosis in young patients are secondary to chronic inflammatory disease. We are reporting a case of a 37-year-old male who was transferred to our hospital for evaluation of possibly acquired bleeding disorder. He was initially presented to an outside hospital with bleeding per rectum for three days duration and one-week history of abdominal pain and bloating. He was found to have nephrotic range proteinuria with hypoalbuminemia and hyperlipidemia. A kidney biopsy was performed to identify the cause of his nephrotic syndrome, and a biopsy showed AL amyloidosis. Bone marrow biopsy performed showed plasma cell myeloma, and the patient was started on induction chemotherapy. Even though the incidence of AL amyloidosis is low before age of 40, we should always perform monoclonal gammopathy workup in patients with nephrotic syndrome regardless of the age. Prompt bone marrow biopsy should be performed to confirm the diagnosis, and starting the treatment as one of the factors that affect the prognosis of AL amyloidosis is early diagnosis.
摘要：
伴有肾脏受累的淀粉样变性是肾病综合征的众所周知的原因。免疫球蛋白轻链淀粉样变性(AL),这是浆细胞损伤在肾脏中单克隆轻链沉积的结果，在40岁之前很少见，通常发生在老年患者身上。大多数年轻患者的肾淀粉样变性病例继发于慢性炎症性疾病。我们正在报告一例37岁男性，他被转移到我们医院评估可能的获得性出血性疾病。他最初被送往医院外，每个直肠出血三天，并有一周的腹痛和腹胀病史。他被发现患有肾病范围蛋白尿，伴有低蛋白血症和高脂血症。进行了肾脏活检以确定肾病综合征的原因，活检显示AL淀粉样变性。骨髓活检显示浆细胞骨髓瘤，患者开始进行诱导化疗。即使在40岁之前AL淀粉样变性的发病率较低，但无论年龄如何，我们都应始终对肾病综合征患者进行单克隆丙种球蛋白病检查。应及时进行骨髓活检以确认诊断，早期诊断是影响AL淀粉样变性预后的因素之一。
