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一例伴有巨噬细胞吞噬单克隆免疫球蛋白λ轻链的毛细血管内增生性肾小球肾炎。

A case of endocapillary proliferative glomerulonephritis with macrophages phagocytosing monoclonal immunoglobulin lambda light chain.

作者信息

Watanabe Hirofumi, Osawa Yutaka, Goto Shin, Habuka Masato, Imai Naofumi, Ito Yumi, Hirose Takayuki, Chou Takaaki, Ohashi Ryuji, Shimizu Akira, Ehara Takashi, Shimotori Takashi, Narita Ichiei

机构信息

Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.

出版信息

Pathol Int. 2015 Jan;65(1):38-42. doi: 10.1111/pin.12229. Epub 2014 Nov 19.

Abstract

Multiple myeloma (MM) is a plasma-cell neoplasm that can cause renal disorders. Renal lesions in MM can present with a very rare pathological manifestation involving a specific monoclonal immunoglobulin (Ig). We report the case of a 33-year-old woman who had edema, fatigue, elevated serum creatinine levels, hypoalbuminemia, and hypercholesterolemia. She had persistent hematuria and proteinuria lasting 3 years. Serum protein electrophoresis showed an M-spike, and serum immunofixation demonstrated the presence of monoclonal IgG λ. She had proteinuria in the nephrotic range, and a monoclonal λ fragment was present on urine immunofixation. Renal biopsy showed proliferative glomerulonephritis with λ light chain and C3c deposition and inflammatory cell infiltration with CD68. Macrophage lysosomes contained λ light chains, suggesting their partial phagocytosis. She was diagnosed with symptomatic MM and was treated with bortezomib and dexamethasone and an autologous peripheral stem cell transplant conditioned with intravenous melphalan. She achieved a partial response with decreased serum monoclonal protein and improved renal function. This case may be categorized as a monoclonal gammopathy-associated proliferative glomerulonephritis. The biopsy finding of partially phagocytosed Ig λ light chains by macrophages is very rare; this pathological condition is similar to crystal-storing histiocytosis.

摘要

多发性骨髓瘤(MM)是一种可导致肾脏疾病的浆细胞瘤。MM中的肾脏病变可表现为一种涉及特定单克隆免疫球蛋白(Ig)的非常罕见的病理表现。我们报告了一例33岁女性病例,该患者有水肿、疲劳、血清肌酐水平升高、低白蛋白血症和高胆固醇血症。她有持续3年的血尿和蛋白尿。血清蛋白电泳显示M峰,血清免疫固定显示存在单克隆IgGλ。她有肾病范围的蛋白尿,尿免疫固定显示存在单克隆λ片段。肾活检显示增殖性肾小球肾炎伴λ轻链和C3c沉积以及CD68阳性的炎性细胞浸润。巨噬细胞溶酶体含有λ轻链,提示部分被吞噬。她被诊断为有症状的MM,并接受了硼替佐米和地塞米松治疗以及以静脉注射美法仑为预处理方案的自体外周干细胞移植。她获得了部分缓解,血清单克隆蛋白减少,肾功能改善。该病例可归类为单克隆丙种球蛋白病相关的增殖性肾小球肾炎。巨噬细胞部分吞噬Igλ轻链的活检发现非常罕见;这种病理状况类似于贮晶组织细胞增多症。

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