Jerez Joaquín, Juanet Cristián, Sharp Joaquín, Ernst Daniel, Méndez Gonzalo P, Sepúlveda Rodrigo A
Departamento de Medicina Interna, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Departamento de Hematología y Oncología, Escuela de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
Rev Med Chil. 2020 Aug;148(8):1059-1067. doi: 10.4067/S0034-98872020000801059.
One of the devastating consequences of monoclonal gammopathies is the development of end-stage kidney disease, which can be prevented with an early diagnosis. Renal involvement can be secondary to saturation of paraproteins with intratubular precipitation or the glomerular deposition of paraproteins with secondary inflammation and destruction. These conditions can also be associated with monoclonal gammopathies that do not meet hematological treatment criteria, called monoclonal gammopathies of renal significance (MGRS).
To report a retrospective analysis of patients who underwent a renal biopsy and whose final diagnosis was a form of monoclonal gammopathy.
We reviewed the clinical and laboratory features and response to treatment of 22 patients aged 63 ± 12 years (55% women) with a pathological diagnosis of a nephropathy associated with paraproteinemia.
The most common hematological diagnosis was amyloidosis in 50% of patients, followed by cast nephropathy. The predominant clinical presentations were proteinuria (without nephrotic syndrome) and nephritic syndrome. Classic criteria such as erythrocyte sedimentation rate > 100 mm/h and protein-albumin gap were unusual. Serum light chain quantification was the test with the best yield to detect paraproteins.
In this group of patients, light chains tend to affect the kidney more commonly than heavy chains. The prognosis of multiple myeloma is much worse than MGRS.
单克隆丙种球蛋白病最严重的后果之一是终末期肾病的发生,早期诊断可预防该病。肾脏受累可能继发于副蛋白在肾小管内沉淀饱和或副蛋白在肾小球沉积并继发炎症和破坏。这些情况也可能与不符合血液学治疗标准的单克隆丙种球蛋白病相关,称为具有肾脏意义的单克隆丙种球蛋白病(MGRS)。
报告对接受肾活检且最终诊断为某种形式单克隆丙种球蛋白病患者的回顾性分析。
我们回顾了22例年龄为63±12岁(55%为女性)、病理诊断为与副蛋白血症相关肾病患者的临床和实验室特征及治疗反应。
最常见的血液学诊断是50%的患者为淀粉样变性,其次是管型肾病。主要临床表现为蛋白尿(无肾病综合征)和肾炎综合征。红细胞沉降率>100mm/h和蛋白 - 白蛋白差值等经典标准并不常见。血清轻链定量是检测副蛋白阳性率最高的检查。
在这组患者中,轻链比重链更易累及肾脏。多发性骨髓瘤的预后比MGRS差得多。