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华氏巨球蛋白血症中的 casts nephropathy 和 light-chain deposition disease。

Cast nephropathy and light-chain deposition disease in Waldenström macroglobulinemia.

机构信息

Department of Pathology, CHU Lille, Lille, France.

出版信息

Am J Kidney Dis. 2012 Sep;60(3):487-91. doi: 10.1053/j.ajkd.2012.01.030. Epub 2012 Jun 20.

Abstract

Waldenström macroglobulinemia is a rare low-grade hematologic malignancy due to clonal proliferation of B lymphocytes responsible for immunoglobulin M (IgM) monoclonal gammopathy secreted in serum. This disease is characterized by lymphoplasmacytic tumoral infiltration of bone marrow and various organs, especially the liver and spleen. Kidney involvement in Waldenström macroglobulinemia has been described previously with reports of various forms of glomerular injury: large intracapillary IgM pseudothrombi, cryoglobulinemia-associated membranoproliferative glomerulonephritis, or amyloidosis. Interstitial infiltration by tumoral B lymphocytes is another classic pattern. Conversely, tubular involvement in the form of myeloma-like casts or basement membrane deposition of monoclonal light chain (light-chain deposition disease) is unusual. We report the occurrence of cast nephropathy associated with light-chain deposition disease in 2 patients with Waldenström macroglobulinemia, which resulted in severe and prolonged kidney failure.

摘要

华氏巨球蛋白血症是一种罕见的低级别血液系统恶性肿瘤,由负责分泌血清中单克隆免疫球蛋白 M(IgM)的 B 淋巴细胞克隆性增殖引起。该病的特征是骨髓和各种器官(尤其是肝和脾)中有淋巴浆细胞肿瘤浸润。以前已有关于华氏巨球蛋白血症肾脏受累的描述,其表现为各种形式的肾小球损伤:大细胞内毛细血管 IgM 假血栓、冷球蛋白血症相关的膜增殖性肾小球肾炎或淀粉样变性。肿瘤性 B 淋巴细胞的间质浸润是另一种经典模式。相反,以骨髓瘤样 casts 或单克隆轻链的基底膜沉积形式(轻链沉积病)的肾小管受累则不常见。我们报告了 2 例华氏巨球蛋白血症患者发生的与轻链沉积病相关的管型肾病,导致严重且持久的肾衰竭。

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