肾移植候选者伴新发现的单克隆伽玛病的胰岛素型淀粉样变性。

IF 0.6 4区 医学 Q4 HEMATOLOGY
Carlos Villarroel, Matthew Howard, Aaron J Wyse, Udit Yadav, Sarah E Gibson
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引用次数: 0

摘要

背景:淀粉样变性是一种异质性疾病,目前已知有42种淀粉样蛋白。最常见的形式包括AL(免疫球蛋白轻链)、ATTR(转甲状腺素)和AA([载脂蛋白]血清淀粉样蛋白A)。除了常规刚果红染色外,淀粉样蛋白分型对于确保淀粉样变患者的适当诊断和临床管理至关重要。目的:在本报告中,我们讨论了基于蛋白质组学的淀粉样蛋白分型在肾移植评估中发现的一种新发现的单克隆γ病(IgG κ pa)患者的应用。本病例强调了识别非al淀粉样蛋白的价值。方法:采用液相色谱串联质谱法(LC-MS/MS)对患者腹腔皮下脂肪抽吸液(SFA)进行淀粉样蛋白鉴定。结果:骨髓活检显示浆细胞骨髓瘤(15% kappa单型浆细胞)。虽然活检的刚果红染色未显示淀粉样蛋白沉积,但在并发SFA中发现了淀粉样蛋白。LC-MS/MS显示的不是AL型淀粉样蛋白,而是AIns(胰岛素)型淀粉样蛋白,证实与患者皮下注射胰岛素有关。有了这些信息,患者被诊断为SMM,并且仍然是肾移植的可行候选人。结论:基于蛋白质组学的淀粉样蛋白分型可以对常见和罕见的淀粉样蛋白类型进行敏感和特异性的鉴定。淀粉样蛋白分型通过这种方法确保适当的临床管理患者的全身和局部淀粉样变。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
AIns (insulin) type amyloidosis in a kidney transplant candidate with a newly identified monoclonal gammopathy.

Background: Amyloidosis is a heterogeneous group of disorders with 42 types of amyloidogenic proteins currently described. The most common forms include AL (immunoglobulin light chain), ATTR (transthyretin), and AA ([Apo] serum amyloid A). Beyond routine Congo red staining, amyloid typing is essential to ensure the appropriate diagnosis and clinical management of patients with amyloidosis.

Purpose: In this report, we discuss the utility of proteomics-based amyloid typing in the setting of a patient with a newly identified monoclonal gammopathy (IgG kappa) discovered during an evaluation for kidney transplantation. This case highlights the value of recognizing non-AL amyloid proteins.

Methods: Amyloid protein identification by liquid chromatography tandem mass spectrometry (LC-MS/MS) was performed on an abdominal subcutaneous fat aspirate (SFA) from the patient.

Results: The bone marrow biopsy demonstrated involvement by plasma cell myeloma (15% kappa monotypic plasma cells). Although a Congo red stain on the biopsy showed no amyloid deposition, amyloid was identified in the concurrent SFA. Instead of AL amyloid, LC-MS/MS demonstrated AIns (insulin) type amyloid, which was confirmed to be related to the patient's subcutaneous insulin injections. With this information, the patient was diagnosed with SMM and remained a viable candidate for kidney transplantation.

Conclusions: Proteomics-based amyloid typing allows sensitive and specific identification of both common and rare amyloid types. Amyloid typing by this method ensures appropriate clinical management of patients with both systemic and localized amyloidosis.

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来源期刊
Journal of Hematopathology
Journal of Hematopathology HEMATOLOGYPATHOLOGY-PATHOLOGY
CiteScore
0.80
自引率
0.00%
发文量
45
期刊介绍: The Journal of Hematopathology aims at providing pathologists with a special interest in hematopathology with all the information needed to perform modern pathology in evaluating lymphoid tissues and bone marrow. To this end the journal publishes reviews, editorials, comments, original papers, guidelines and protocols, papers on ancillary techniques, and occasional case reports in the fields of the pathology, molecular biology, and clinical features of diseases of the hematopoietic system. The journal is the unique reference point for all pathologists with an interest in hematopathology. Molecular biologists involved in the expanding field of molecular diagnostics and research on lymphomas and leukemia benefit from the journal, too. Furthermore, the journal is of major interest for hematologists dealing with patients suffering from lymphomas, leukemias, and other diseases. The journal is unique in its true international character. Especially in the field of hematopathology it is clear that there are huge geographical variations in incidence of diseases. This is not only locally relevant, but due to globalization, relevant for all those involved in the management of patients.
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