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揭示一种罕见的椎管狭窄病因:并存的轻链型和转甲状腺素蛋白型淀粉样变性累及黄韧带。

Unraveling a rare cause of spinal stenosis: Coexistent AL and ATTR amyloidosis involving the ligamentum flavum.

作者信息

Godara Amandeep, Wang Andy Y, Arkun Knarik, Fogaren Teresa, Qamar Adnan S, McPhail Ellen D, Kryzanski James, Riesenburger Ron, Comenzo Raymond

机构信息

Department of Hematology and Hematologic Malignancies, University of Utah, Salt Lake, Utah.

Department of Neurosurgery, Tufts Medical Center, Washington, Boston, United States.

出版信息

Surg Neurol Int. 2022 Jan 12;13:12. doi: 10.25259/SNI_1235_2021. eCollection 2022.

Abstract

BACKGROUND

Amyloidosis is a protein misfolding disorder that leads to the deposition of beta-pleated sheets of a fibrillar derivative of various protein precursors. Identification of the type of precursor protein is integral in treatment decision-making. The presence of two different types of amyloid in the same patient is unusually rare, and there are no previous reports of two different types of amyloid deposition in the ligamentum flavum (LF) in the same patient.

CASE DESCRIPTION

Here, we describe two patients with spinal stenosis who underwent laminectomies and were found to have AL and ATTR amyloid deposits in the LF.

CONCLUSION

As the spine is becoming recognized as a site for ATTRwt amyloid deposition, patients undergoing spinal decompression surgery may potentially benefit from evaluation for amyloidosis in the LF.

摘要

背景

淀粉样变性是一种蛋白质错误折叠疾病,可导致各种蛋白质前体的纤维状衍生物的β折叠片层沉积。确定前体蛋白的类型对于治疗决策至关重要。同一患者体内存在两种不同类型的淀粉样蛋白极为罕见,此前尚无同一患者黄韧带(LF)中出现两种不同类型淀粉样蛋白沉积的报道。

病例描述

在此,我们描述了两名接受椎板切除术的腰椎管狭窄症患者,其黄韧带中发现有AL和ATTR淀粉样蛋白沉积。

结论

由于脊柱正被认为是野生型转甲状腺素蛋白淀粉样变性(ATTRwt)沉积的部位,接受脊柱减压手术的患者可能会受益于对黄韧带淀粉样变性的评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/cbe0/8813631/d151bcfe5f2e/SNI-13-12-g001.jpg

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