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一名复发多发性骨髓瘤患者近端肾小管病无结晶的超微结构鉴定

Ultrastructural identification of a proximal tubulopathy without crystals in a relapsed multiple myeloma patient.

作者信息

Brealey John K, Tran Yung, Ninnes Ruth, Abeyaratne Asanga

机构信息

a Division of Anatomical Pathology , SA Pathology , Adelaide , Australia.

b Renal Unit , Royal Darwin Hospital , Darwin , Australia.

出版信息

Ultrastruct Pathol. 2018 Sep-Oct;42(5):458-463. doi: 10.1080/01913123.2018.1526243. Epub 2018 Sep 25.

DOI:10.1080/01913123.2018.1526243
PMID:30252563
Abstract

A multiple myeloma patient, who had been treated with a hematopoietic stem cell transplant, underwent a renal biopsy for investigation of a possible relapse of disease as indicated by increased serum creatinine and positive urinary Bence-Jones protein containing increased kappa light chain. Paraprotein-related renal disease was not evident by light microscopy or immunofluorescence microscopy however electron microscopy demonstrated a proximal tubulopathy with intracytoplasmic non-crystalline inclusions. The ultrastructural findings suggested possible end-organ involvement by the disease and follow-up studies subsequently revealed a relapsed multiple myeloma in the patient. The case exemplifies the usefulness of electron microscopy in detecting paraproteins that, in some instances, may be difficult to demonstrate by other techniques.

摘要

一名接受过造血干细胞移植治疗的多发性骨髓瘤患者,因血清肌酐升高及尿本-周蛋白阳性(含κ轻链增加)提示可能疾病复发而接受肾活检。光镜或免疫荧光显微镜检查未发现副蛋白相关肾病,但电镜显示近端肾小管病伴胞质内非结晶性包涵体。超微结构发现提示疾病可能累及终末器官,随后的随访研究显示该患者多发性骨髓瘤复发。该病例证明了电镜在检测副蛋白方面的有用性,在某些情况下,其他技术可能难以检测到这些副蛋白。

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引用本文的文献

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Non-crystalline light chain proximal tubulopathy, a morphologically protean entity.非结晶轻链近端肾小管病,一种形态多样的实体。
Nephrol Dial Transplant. 2023 Oct 31;38(11):2576-2588. doi: 10.1093/ndt/gfad085.
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Light Chain Restriction in Proximal Tubules-Implications for Light Chain Proximal Tubulopathy.近端小管中的轻链限制——对轻链近端小管病的影响。
Front Med (Lausanne). 2022 Mar 28;9:723758. doi: 10.3389/fmed.2022.723758. eCollection 2022.