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具有肾意义的单克隆丙种球蛋白病中的重链沉积病:1例多发性骨髓瘤前驱病例及文献综述

Heavy Chain Deposition Disease in Monoclonal Gammopathy of Renal Significance: A Prodrome of Multiple Myeloma Case and Literature Review.

作者信息

Gnanasampanthan Sahana, Samelis Vasileios P, Roufosse Candice, Kousios Andreas

机构信息

Renal Medicine, Royal London Hospital, Barts Health NHS Trust, London, GBR.

Medicine, School of Medicine, European University of Cyprus, Nicosia, CYP.

出版信息

Cureus. 2025 Jun 10;17(6):e85740. doi: 10.7759/cureus.85740. eCollection 2025 Jun.

DOI:10.7759/cureus.85740
PMID:40656361
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12246656/
Abstract

Monoclonal immunoglobulin deposition disease (MIDD) is a complication of plasma cell dyscrasias, resulting in abnormal immunoglobulin deposition along basement membranes. We describe a case of a 60-year-old male with a complex hospital admission, presenting with critical illness accompanied by acute kidney injury, nephrotic syndrome and moderately elevated serum free light chain (SFLC) ratio, on a background of well-controlled diabetes, hypertension and chronic kidney disease. There was no clear aetiology for his presentation following preliminary examination and investigations, which led to a biopsy diagnosis of heavy chain deposition disease (HCDD) in the context of monoclonal gammopathy of renal significance (MGRS). We explore the importance of understanding the disease course to allow timely biopsy diagnosis and treatment initiation. Our patient required very close follow-up and a wide multi-disciplinary approach, including haematologists, nephrologists and histopathologists, to guide management in a disease for which therapeutic strategies are poorly defined due to limited clinical trial data.

摘要

单克隆免疫球蛋白沉积病(MIDD)是浆细胞异常增生的一种并发症,可导致免疫球蛋白沿基底膜异常沉积。我们描述了一例60岁男性复杂的住院病例,该患者以危重症入院,伴有急性肾损伤、肾病综合征以及血清游离轻链(SFLC)比值中度升高,其基础疾病为控制良好的糖尿病、高血压和慢性肾脏病。初步检查和调查后,其病情的病因尚不明确,最终在具有肾脏意义的单克隆丙种球蛋白病(MGRS)背景下,经活检诊断为重链沉积病(HCDD)。我们探讨了了解疾病病程对于及时进行活检诊断和开始治疗的重要性。我们的患者需要密切随访,并采用多学科综合方法,包括血液科医生、肾内科医生和组织病理学家,以指导对这种因临床试验数据有限而治疗策略尚不明确的疾病的管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/a504cbdd57a9/cureus-0017-00000085740-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/d0bb52940b85/cureus-0017-00000085740-i01.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/20de899e7cdc/cureus-0017-00000085740-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/39816164053d/cureus-0017-00000085740-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/a504cbdd57a9/cureus-0017-00000085740-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/d0bb52940b85/cureus-0017-00000085740-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/90bf199f3d56/cureus-0017-00000085740-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/20de899e7cdc/cureus-0017-00000085740-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/39816164053d/cureus-0017-00000085740-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b24/12246656/a504cbdd57a9/cureus-0017-00000085740-i05.jpg

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本文引用的文献

1
Heavy Chain Deposition Disease: Clinicopathologic Characteristics of a Chinese Case Series.重链沉积病:中国病例系列的临床病理特征。
Am J Kidney Dis. 2020 May;75(5):736-743. doi: 10.1053/j.ajkd.2019.08.013. Epub 2019 Nov 5.
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Randall-type monoclonal immunoglobulin deposition disease: novel insights from a nationwide cohort study.兰德尔型单克隆免疫球蛋白沉积病:全国性队列研究的新见解。
Blood. 2019 Feb 7;133(6):576-587. doi: 10.1182/blood-2018-09-872028. Epub 2018 Dec 21.
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Hematologic and renal improvement of monoclonal immunoglobulin deposition disease after treatment with bortezomib-based regimens.
基于硼替佐米方案治疗后单克隆免疫球蛋白沉积病的血液学和肾脏改善情况
Leuk Lymphoma. 2017 Aug;58(8):1832-1839. doi: 10.1080/10428194.2016.1267349. Epub 2016 Dec 14.
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Outcomes of patients with renal monoclonal immunoglobulin deposition disease.肾单克隆免疫球蛋白沉积病患者的结局。
Am J Hematol. 2016 Nov;91(11):1123-1128. doi: 10.1002/ajh.24528. Epub 2016 Aug 29.
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Natural history and outcome of light chain deposition disease.轻链沉积病的自然病程与转归
Blood. 2015 Dec 24;126(26):2805-10. doi: 10.1182/blood-2015-07-658872. Epub 2015 Sep 21.
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Bortezomib produces high hematological response rates with prolonged renal survival in monoclonal immunoglobulin deposition disease.硼替佐米治疗单克隆免疫球蛋白沉积病可产生较高的血液学缓解率并延长肾脏生存时间。
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Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant.肾脏相关意义的单克隆丙种球蛋白病:当 MGUS 不再是不确定或无意义的时候。
Blood. 2012 Nov 22;120(22):4292-5. doi: 10.1182/blood-2012-07-445304. Epub 2012 Oct 9.
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Renal monoclonal immunoglobulin deposition disease: a report of 64 patients from a single institution.肾脏单克隆免疫球蛋白沉积病:单中心 64 例报告。
Clin J Am Soc Nephrol. 2012 Feb;7(2):231-9. doi: 10.2215/CJN.08640811. Epub 2011 Dec 8.
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