Gödecke V, Schmidt J J, Bräsen J H, Koenecke C, Haller H
Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
Institut für Pathologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.
Internist (Berl). 2019 Jan;60(1):10-22. doi: 10.1007/s00108-018-0538-7.
Kidney involvement is a common complication in patients with plasma cell diseases.
This article outlines the spectrum of renal involvement in plasma cell dyscrasia and describes diagnostic and therapeutic measures to guide clinical management.
Evaluation and discussion of the current literature as well as existing guidelines and recommendations of professional societies.
The clinical manifestations of renal involvement in plasma cell disorders are heterogeneous and range from acute cast nephropathy in multiple myeloma to rare forms of glomerulonephritis. The term monoclonal gammopathy of renal significance (MGRS) was introduced to describe kidney involvement caused by monoclonal gammopathy but without evidence for underlying malignancy. Light chain cast nephropathy is the most common renal manifestation in multiple myeloma, whereas monoclonal immunoglobulin deposition disease (MIDD) and renal light chain (AL) amyloidosis can be found in multiple myeloma and MGRS. Decisive is the extended hematological diagnostics in order to exclude the presence of a hematological neoplasm. The treatment of renal involvement in monoclonal gammopathies involves the reduction of the plasma cell clone with cytoreductive treatment. The reduction of the monoclonal protein in serum is prognostically relevant for the renal response to treatment. In the case of histological evidence of a light chain cast nephropathy, high cut-off dialysis is recommended to reduce the free light chains in serum.
The spectrum of renal manifestations in plasma cell dyscrasia has been expanded, particularly since the introduction of the term MGRS. Diagnostic and therapeutic management remain an interdisciplinary challenge.
肾脏受累是浆细胞疾病患者常见的并发症。
本文概述浆细胞发育异常中肾脏受累的范围,并描述指导临床管理的诊断和治疗措施。
对当前文献以及专业学会的现有指南和建议进行评估与讨论。
浆细胞疾病肾脏受累的临床表现具有异质性,范围从多发性骨髓瘤中的急性管型肾病到罕见的肾小球肾炎形式。引入了具有肾脏意义的单克隆丙种球蛋白病(MGRS)这一术语来描述由单克隆丙种球蛋白病引起但无潜在恶性肿瘤证据的肾脏受累情况。轻链管型肾病是多发性骨髓瘤中最常见的肾脏表现,而单克隆免疫球蛋白沉积病(MIDD)和肾脏轻链(AL)淀粉样变性可见于多发性骨髓瘤和MGRS。关键在于进行全面的血液学诊断以排除血液系统肿瘤的存在。单克隆丙种球蛋白病肾脏受累的治疗包括通过细胞减灭治疗减少浆细胞克隆。血清中单克隆蛋白的减少对肾脏治疗反应的预后具有相关性。在有轻链管型肾病组织学证据的情况下,建议进行高通量透析以降低血清中的游离轻链。
浆细胞发育异常中肾脏表现的范围已经扩大,特别是自MGRS这一术语引入以来。诊断和治疗管理仍然是一个跨学科的挑战。