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伴有轻链沉积病的假性非分泌性多发性骨髓瘤

Pseudo-nonsecretory multiple myeloma with light chain deposition disease.

作者信息

Matsuzaki H, Yoshida M, Akahoshi Y, Kuwahara K, Satou T, Takatsuki K

机构信息

Second Department of Internal Medicine, Kumamoto University Medical School, Japan.

出版信息

Acta Haematol. 1991;85(3):164-8. doi: 10.1159/000204882.

Abstract

A diagnosis of nonsecretory myeloma was established in two patients with anemia and proteinuria on the basis of the suppression of polyclonal immunoglobulins and the increase of plasma cells in the bone marrow. No paraprotein was detected in the serum or concentrated urine of these patients. However, a plaque-forming assay of bone marrow cells showed the secretion of monoclonal immunoglobulin by the myeloma cells. Moreover, renal biopsies from both patients indicated the deposition of monoclonal light chains in the glomerular mesangium and basement membrane, as well as in the tubular basement membrane, a pattern consistent with light-chain deposition disease. These observations suggested that the secreted paraprotein disappeared rapidly as a result of enhanced catabolism or deposition in organs such as the kidney, producing severe proteinuria and chronic renal failure. The plaque-forming assay is a useful technique for the demonstration of this type of nonsecretory myeloma, pseudo-nonsecretory myeloma.

摘要

两名患有贫血和蛋白尿的患者被诊断为非分泌型骨髓瘤,诊断依据是多克隆免疫球蛋白受到抑制以及骨髓中浆细胞增多。在这些患者的血清或浓缩尿中未检测到副蛋白。然而,对骨髓细胞进行的空斑形成试验显示骨髓瘤细胞分泌单克隆免疫球蛋白。此外,两名患者的肾活检均表明单克隆轻链沉积于肾小球系膜和基底膜以及肾小管基底膜,这种模式与轻链沉积病一致。这些观察结果表明,分泌的副蛋白由于分解代谢增强或沉积于肾脏等器官而迅速消失,从而导致严重蛋白尿和慢性肾衰竭。空斑形成试验是用于证实这类非分泌型骨髓瘤即假性非分泌型骨髓瘤的一项有用技术。

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