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一名37岁患者因轻链淀粉样变性导致肾病综合征的罕见病例。

Unusual Case of Nephrotic Syndrome From Light Chain Amyloidosis in a 37-Year-Old Patient.

作者信息

Salameh Omar K, Darok Matthew C, Kane Jennifer A, Abendroth Catherine, Trivedi Naman

机构信息

Medicine/Nephrology, Penn State Health Milton S. Hershey Medical Center, Hershey, USA.

College of Medicine, Penn State College of Medicine, Hershey, USA.

出版信息

Cureus. 2021 Sep 20;13(9):e18120. doi: 10.7759/cureus.18120. eCollection 2021 Sep.

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

肾受累的淀粉样变性是肾病综合征的一个众所周知的病因。免疫球蛋白轻链淀粉样变性(AL)是浆细胞异常增生导致单克隆轻链在肾脏沉积的结果,在40岁之前较为罕见,通常发生于老年患者。年轻患者的肾淀粉样变性大多继发于慢性炎症性疾病。我们报告一例37岁男性患者,他因评估可能获得性出血性疾病转至我院。他最初因直肠出血3天、腹痛和腹胀1周就诊于外院。发现他有肾病范围蛋白尿伴低白蛋白血症和高脂血症。进行肾活检以确定其肾病综合征的病因,活检显示为AL淀粉样变性。骨髓活检显示为浆细胞骨髓瘤,患者开始接受诱导化疗。尽管AL淀粉样变性在40岁之前发病率较低,但对于肾病综合征患者,无论年龄大小,我们都应始终进行单克隆丙种球蛋白病检查。应及时进行骨髓活检以确诊,并且尽早开始治疗是影响AL淀粉样变性预后的因素之一,因为早期诊断很重要。

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