Lambda light chain - restricted non - crystalline proximal tubulopathy with cast nephropathy in multiple myeloma: a case report and literature review.

IF 2.2 4区 医学 Q2 UROLOGY & NEPHROLOGY
Mingfu Lan, Yaohui Guo, Caiyun Wang, Xiaoqin Wang, Jing Li, Yanxia Wang
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Abstract

Background: Multiple myeloma (MM) often causes renal tubular damage, such as the light chain cast nephropathy (LCCN) and the light chain proximal tubulopathy (LCPT). The excessive light chains deposited in the proximal and distal tubules usually manifest with different characteristics, leading to a rare coexistence of the two pathological conditions. Here we report a unique case of a patient with multiple myeloma (MM) who presented with acute kidney injury (AKI) due to dual conditions of λ light chain-restricted non-crystalline LCPT and LCCN. This report reviews the clinical presentation and histological findings, comparing them with previously published cases.

Case presentation: A 49-year-old male patient was admitted with a chief complaint of "fatigue, loss of appetite for 40 days and elevated blood creatinine for 10 days." In serum and urine, the λ light chain level and the ratio of κ to λ free light chain were 1235 mg/dl and 93.25 mg/dl, 0.0022 and 0.0316, respectively. Additionally, serum protein electrophoresis showed an M-spike with monoclonal IgD-λ. Bone marrow puncture revealed 30.5% primitive naive plasma cells, indicative of IgD-λ MM. Light microscopy of kidney biopsy specimen showed periodic acid-Schiff (PAS)-negative cytoplasm in some proximal tubules and PAS-negative casts with a rigid appearance in some distal tubule lumens. On immunofluorescence, these proximal tubular epithelial cells cytoplasm and casts stained exclusively with λ-light chains. Electron microscopy did not reveal any crystalline inclusions. Given the clinical and bone marrow puncture findings, the overall pathological presentation was LCPT with LCCN secondary to IgD-λ MM. After chemotherapy and dialysis, the patient's condition was improved and he was tracked in follow-ups.

Conclusion: In some tubular renal injuries caused by MM, the morphological changes are subtle and often overlooked. In this paper, we present a rare case of LCPT with LCCN showing λ restriction in patient with MM. Through the clinicopathological analysis of patients, the understanding of the disease can be deepened and the diagnosis rate improved.

多发性骨髓瘤伴铸型肾病的λ轻链限制性非结晶近端肾小管病变:病例报告和文献综述。
背景:多发性骨髓瘤(MM)经常引起肾小管损伤,如轻链铸型肾病(LCCN)和轻链近端肾小管病变(LCPT)。沉积在近端肾小管和远端肾小管的过量轻链通常表现出不同的特征,导致这两种病症罕见地同时存在。在此,我们报告了一例独特的多发性骨髓瘤(MM)患者,该患者因同时患有λ轻链限制性非结晶性LCPT和LCCN而出现急性肾损伤(AKI)。本报告回顾了该病例的临床表现和组织学检查结果,并与之前发表的病例进行了比较:一名 49 岁男性患者入院,主诉为 "乏力、食欲不振 40 天,血肌酐升高 10 天"。血清和尿液中的λ轻链水平以及κ与λ游离轻链的比值分别为 1235 mg/dl 和 93.25 mg/dl, 0.0022 和 0.0316。此外,血清蛋白电泳显示存在单克隆 IgD-λ 的 M 峰。骨髓穿刺显示有30.5%的原始幼稚浆细胞,表明存在IgD-λ MM。肾活检标本的光学显微镜检查显示,一些近端肾小管的细胞质呈周期性酸-Schiff(PAS)阴性,一些远端肾小管管腔的细胞质呈PAS阴性,外观僵硬。免疫荧光显示,这些近端肾小管上皮细胞的细胞质和铸型完全被λ-轻链染色。电子显微镜检查未发现任何晶体内含物。鉴于临床和骨髓穿刺结果,总体病理表现为继发于 IgD-λ MM 的 LCPT 伴 LCCN。经过化疗和透析,患者病情有所好转,并接受了随访:结论:在一些由 MM 引起的肾小管损伤中,其形态学变化很微妙,常常被忽视。本文介绍了一例罕见的LCPT伴LCCN显示λ受限的MM患者。通过对患者的临床病理分析,可以加深对该病的认识,提高诊断率。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
BMC Nephrology
BMC Nephrology UROLOGY & NEPHROLOGY-
CiteScore
4.30
自引率
0.00%
发文量
375
审稿时长
3-8 weeks
期刊介绍: BMC Nephrology is an open access journal publishing original peer-reviewed research articles in all aspects of the prevention, diagnosis and management of kidney and associated disorders, as well as related molecular genetics, pathophysiology, and epidemiology.
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