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多发性骨髓瘤中的肾脏病变:它们与相关蛋白质异常的关系。

Renal lesions in multiple myeloma: their relationship to associated protein abnormalities.

作者信息

Hill G S, Morel-Maroger L, Méry J P, Brouet J C, Mignon F

出版信息

Am J Kidney Dis. 1983 Jan;2(4):423-38. doi: 10.1016/s0272-6386(83)80075-4.

DOI:10.1016/s0272-6386(83)80075-4
PMID:6823960
Abstract

Renal biopsy and autopsy specimens were studied in 43 patients with renal complications of multiple myeloma and correlated with immunoelectrophoresis (IEP) and other clinical data at the time of biopsy. Lesions specifically related to multiple myeloma fell into two categories, with different patterns of protein excretion. (1) Myeloma Cast Formation: When other lesions which might contribute to renal insufficiency (RI) were excluded, there was a good correlation between the extent of myeloma cast formation and severity of RI. Sixteen of 19 patients excreted free light chains (LCs) in the urine, in seven as the predominant or sole urinary protein. (2) Tissue Deposition of Paraproteins: Nine cases had generalized glomerular, tubular basement membrane and vascular deposits of presumed kappa-chains (one with associated alpha-heavy chains). Four patients, all with myelomas secreting lambda LCs, had diffuse amyloid deposits in similar distribution. All patients (save two who were anuric) had diffuse, nonselective proteinuria by IEP, most within the nephrotic range. Four patients had free LCs in the urine, but in none was this the predominant component. Cast nephropathy and LC tissue deposition tended to occur in mutually exclusive fashion. Cases with diffuse tissue deposits of LCs showed few or no myeloma casts. Cases with cast nephropathy had only occasional mild mesangial lesions and focal interstitial and vascular deposits of amyloid. Evidence indicates that these lesions represent incidental LC deposition in cases whose basic lesion is longstanding and/or severe cast nephropathy, and that their contribution to RI is minor in comparison to that of the myeloma casts.

摘要

对43例多发性骨髓瘤肾并发症患者的肾活检和尸检标本进行了研究,并与活检时的免疫电泳(IEP)及其他临床资料进行了相关性分析。与多发性骨髓瘤特异性相关的病变分为两类,蛋白排泄模式不同。(1)骨髓瘤管型形成:排除可能导致肾功能不全(RI)的其他病变后,骨髓瘤管型形成的程度与RI的严重程度之间存在良好的相关性。19例患者中有16例尿中排泄游离轻链(LCs),其中7例以游离轻链为主要或唯一尿蛋白。(2)副蛋白的组织沉积:9例患者肾小球、肾小管基底膜和血管出现推测为κ链的广泛沉积(1例伴有α重链)。4例均分泌λ LC的骨髓瘤患者,在相似分布部位有弥漫性淀粉样沉积。所有患者(除2例无尿者外)经IEP检测均有弥漫性、非选择性蛋白尿,多数在肾病范围内。4例患者尿中有游离LCs,但均不是主要成分。管型肾病和LC组织沉积往往以相互排斥的方式出现。LCs有弥漫性组织沉积的病例几乎没有或没有骨髓瘤管型。有管型肾病的病例仅有偶尔的轻度系膜病变以及局灶性间质和血管淀粉样沉积。有证据表明,这些病变代表在基本病变为长期存在和/或严重管型肾病的病例中偶然发生的LC沉积,与骨髓瘤管型相比,它们对RI的影响较小。

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Indian J Nephrol. 2024 Jan-Feb;34(1):31-36. doi: 10.4103/ijn.ijn_362_22. Epub 2023 Jun 5.
2
Tubular basement membrane amyloid deposition: is it an indicator of renal progression in light chain amyloidosis?管状基底膜淀粉样物质沉积:它是否是轻链淀粉样变性患者肾脏进展的一个指标?
Ren Fail. 2023 Dec;45(1):2203776. doi: 10.1080/0886022X.2023.2203776.
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Two kinds of rare light chain cast nephropathy caused by multiple myeloma: case reports and literature review.
两种由多发性骨髓瘤引起的罕见轻链 casts 肾病:病例报告和文献复习。
BMC Nephrol. 2021 Jan 28;22(1):42. doi: 10.1186/s12882-021-02250-z.
4
Multiple morphological phenotypes of monoclonal immunoglobulin disease on renal biopsy: Significance of treatment.肾活检中单克隆免疫球蛋白病的多种形态学表型:治疗的意义
Clin Nephrol Case Stud. 2020 Apr 17;8:17-24. doi: 10.5414/CNCS110052. eCollection 2020.
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Intratubular amyloid in light chain cast nephropathy is a risk factor for systemic light chain amyloidosis.轻链 casts 肾病中的管内淀粉样变是系统性轻链淀粉样变性的一个危险因素。
Mod Pathol. 2018 Mar;31(3):452-462. doi: 10.1038/modpathol.2017.124. Epub 2017 Oct 20.
6
Antineutrophil Cytoplasmic Antibodies-Negative Pauci-Immune Crescentic Glomerulonephritis Associated with Multiple Myeloma.抗中性粒细胞胞浆抗体阴性的寡免疫性新月体性肾小球肾炎合并多发性骨髓瘤
Case Rep Nephrol Dial. 2015 Jun 4;5(2):124-9. doi: 10.1159/000432394. eCollection 2015 May-Aug.
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Clin Exp Nephrol. 2011 Aug;15(4):586-90. doi: 10.1007/s10157-011-0437-x. Epub 2011 Mar 25.
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Med Oncol. 2011 Mar;28(1):199-201. doi: 10.1007/s12032-009-9404-8. Epub 2010 Jan 5.
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