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[多发性骨髓瘤的肾脏受累。病理生理学与治疗]

[Renal involvement in multiple myeloma. Physiopathology and therapy].

作者信息

Fiorini F, Napoli S, Giordano L, Castelluccio A

机构信息

Bussana di Sanremo (Imperia), Servizio Dialisi.

出版信息

Minerva Urol Nefrol. 1995 Sep;47(3):125-31.

PMID:8815549
Abstract

Renal failure (RF) is a common accompaniment of multiple myeloma and is identified in over half of patients at presentation. RF is usually related to the presence of Bence-Jones protein (immunoglobulin light chain) which damages all the compartments of the kidney: glomerule, tubulo-interstitium and vasculature. The most common renal lesion is cast nephropathy, named "myeloma kidney": Cast are produced by two mechanisms: proximal tubule damage and intratubular cast formation. The predominant pathophysiologic mechanism of tubule damage appears to be a precipitation of Bence-Jones protein and Tamm-Horsfall glycoprotein produced by cells of ascending limb of Henle's loop in the tubule lumen. The therapeutic maneuvers to reduce renal damage and preserve renal function are reduction of plasma concentration of light chain with chemotherapy, elimination of factors which favour coprecipitation of Tamm-Horsfall protein with light chain (hypercalcemia, acid urine, radiocontrast material, furosemide, oliguria). At last, colchicine (1.2 mg/day) will also be efficacious in the acute management of patients with cast nephropathy.

摘要

肾衰竭(RF)是多发性骨髓瘤的常见伴随病症,超过半数的患者在就诊时即被确诊。RF通常与本周氏蛋白(免疫球蛋白轻链)的存在有关,该蛋白会损害肾脏的所有结构:肾小球、肾小管间质和血管。最常见的肾脏病变是管型肾病,即“骨髓瘤肾病”:管型的形成有两种机制:近端小管损伤和肾小管内管型形成。肾小管损伤的主要病理生理机制似乎是亨氏袢升支细胞产生的本周氏蛋白和Tamm-Horsfall糖蛋白在肾小管腔内沉淀。减少肾脏损伤和保护肾功能的治疗措施包括通过化疗降低轻链的血浆浓度,消除有利于Tamm-Horsfall蛋白与轻链共沉淀的因素(高钙血症、酸性尿液、放射性造影剂、呋塞米、少尿)。最后,秋水仙碱(1.2毫克/天)对管型肾病患者的急性治疗也有效。

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