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多发性骨髓瘤合并肝淀粉样变性轻链沉积病,表现为进行性肝功能衰竭。

Multiple myeloma with hepatic amyloid light-chain amyloidosis manifesting as progressive liver failure.

机构信息

Internal Medicine, University of Florida Health at Jacksonville, Jacksonville, Florida, USA

Internal Medicine, University of Florida Health at Jacksonville, Jacksonville, Florida, USA.

出版信息

BMJ Case Rep. 2020 Jun 29;13(6):e234637. doi: 10.1136/bcr-2020-234637.

Abstract

We present a case of a 45-year-old man admitted to the hospital with new-onset ascites and bilateral subconjunctival haemorrhages. He was found to have elevated liver enzymes in a hepatocellular pattern and direct hyperbilirubinemia. A diagnostic paracentesis was consistent with portal hypertension (PH). Extensive workup for acute and chronic liver disease was unremarkable. In the absence of clinical evidence of cirrhosis to explain PH, a liver biopsy with hepatic venous pressure gradient was pursued, which revealed proteinaceous material and apple-green birefringence under polarised light consistent with amyloid deposits. Bone marrow biopsy revealed plasma cell neoplasm with proteinaceous deposits consistent with concomitant multiple myeloma with AL amyloidosis. He developed rapidly progressive liver failure and passed shortly after presentation despite treatment with chemotherapy. This case illustrates how primary hepatic amyloidosis can present with a physiology that mimics cirrhosis and can easily be missed.

摘要

我们报告一例 45 岁男性,因新发腹水和双侧球结膜下出血住院。他的肝酶呈肝细胞模式升高,直接胆红素升高。诊断性腹腔穿刺术提示门静脉高压(PH)。对急性和慢性肝病的广泛检查无明显异常。由于没有临床证据表明肝硬化可以解释 PH,因此进行了肝活检和肝静脉压力梯度测量,结果显示蛋白样物质和偏光下的苹果绿双折射,符合淀粉样变沉积。骨髓活检显示浆细胞瘤和蛋白样沉积物,符合同时发生的多发性骨髓瘤伴 AL 淀粉样变性。尽管接受了化疗,但他很快出现进行性肝功能衰竭并在就诊后不久死亡。本例说明了原发性肝淀粉样变性如何表现出类似于肝硬化的生理学特征,并且很容易被忽视。

相似文献

本文引用的文献

1
Acquired Factor X Deficiency in Patients With Primary Light Chain Amyloidosis.原发性轻链淀粉样变性患者获得性因子X缺乏症
J Investig Med High Impact Case Rep. 2019 Jan-Dec;7:2324709619832332. doi: 10.1177/2324709619832332.
6
Amyloidosis: an unusual cause of portal hypertension.淀粉样变性:门静脉高压的一种罕见病因。
Autops Case Rep. 2016 Jun 30;6(2):9-18. doi: 10.4322/acr.2016.035. eCollection 2016 Apr-Jun.

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