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[一例IgD型骨髓瘤合并急性肾衰竭及λ轻链沿肾小管基底膜沉积的病例报告]

[A case report of IgD myeloma with acute renal failure and deposition of lambda light chain along the tubular basement membrane].

作者信息

Takeda A, Morozumi K, Yoshida A, Suganuma T, Satoh K, Oikawa T, Fujinami T, Sigematsu H

出版信息

Nihon Jinzo Gakkai Shi. 1989 Mar;31(3):259-66.

PMID:2500549
Abstract

A case of IgD lambda type multiple myeloma that developed acute renal failure was presented. Histopathologic studies revealed apparent myeloma cast nephropathy with typical giant cells and light chain deposition along tubular basement membrane and inter-peritubular interstitium. Glomerular deposits of light chain were absent by electron and immunofluorescent microgram. The presented case showed a very uncommon histopathologic findings of unusual deposits of light chain, to tubulo-interstitium except for glomeruli, in addition to vertically rare IgD myeloma. A sixty-year old female who was suffered from acute renal failure and treated by hemodialysis for one month was admitted to our institute. Physical examinations revealed ill-apparent and emaciated uremic patient on admission. Laboratory and radiological studies demonstrated IgD lambda type monoclonal protein and numerous punched out appearance of skull bone. The diagnosis of IgD lambda myeloma was confirmed with aspiration bone marrow test. Anti-myeloma therapy of melphalan and prednisolone have started, however, fatal arrhythmia suddenly suffered the patient and resuscitation attempts failed to get any success one week after admission. Renal necropsy was performed and specimens were studied by light, immunofluorescent and electron microscope.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

本文报告1例发生急性肾衰竭的IgD λ型多发性骨髓瘤。组织病理学研究显示明显的骨髓瘤管型肾病,伴有典型巨细胞,轻链沿肾小管基底膜和肾小管周围间质沉积。电子显微镜和免疫荧光显微镜检查未发现轻链在肾小球沉积。该病例除了极罕见的IgD骨髓瘤外,还表现出非常罕见的组织病理学特征,即轻链在除肾小球外的肾小管间质异常沉积。一名60岁女性因急性肾衰竭接受血液透析治疗1个月后入住我院。入院体格检查显示患者面容憔悴、消瘦且有尿毒症表现。实验室和影像学检查显示IgD λ型单克隆蛋白以及颅骨多处穿凿样表现。经骨髓穿刺检查确诊为IgD λ型骨髓瘤。开始使用美法仑和泼尼松进行抗骨髓瘤治疗,然而,患者在入院1周后突然发生致命性心律失常,复苏尝试未成功。进行了肾脏尸检,并对标本进行了光镜、免疫荧光和电子显微镜检查。(摘要截短至250字)

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