Ventura Sofia, Cabral Raquel, Viveiros Carolina, S Santos Mariana, Esteves João
Nephrology, Hospital do Divino Espírito Santo, EPER, Ponta Delgada, PRT.
Hematology, Hospital do Divino Espírito Santo, EPER, Ponta Delgada, PRT.
Cureus. 2022 Dec 20;14(12):e32753. doi: 10.7759/cureus.32753. eCollection 2022 Dec.
Monoclonal gammopathy of renal significance (MGRS) is a group of pathologies that includes all kidney disorders related to a monoclonal protein in patients without diagnostic criteria for B-cell malignancies. There are multiple MGRS-associated kidney disorders, and more are still being discovered, which makes this diagnosis challenging. The relationship between monoclonal gammopathies and thrombotic microangiopathy (TMA) is of growing interest in literature. This article describes the case of a patient with newly diagnosed MGRS, presenting with rapidly progressing kidney failure and with histologic characteristics of TMA. The patient progressed to end-stage renal disease (ESRD) despite treatment with plasmapheresis and clone-directed therapy, as is currently advised in the literature. Although rare, the association between these two entities should not be unnoticed because of patients' renal and vital prognosis.
具有肾意义的单克隆丙种球蛋白病（MGRS）是一组病症，包括所有与单克隆蛋白相关的肾脏疾病，这些患者无B细胞恶性肿瘤的诊断标准。有多种与MGRS相关的肾脏疾病，而且仍有更多疾病正在被发现，这使得该诊断具有挑战性。单克隆丙种球蛋白病与血栓性微血管病（TMA）之间的关系在文献中越来越受到关注。本文描述了一例新诊断为MGRS的患者，表现为快速进展的肾衰竭且具有TMA的组织学特征。尽管按照目前文献建议采用了血浆置换和克隆定向治疗，但该患者仍进展为终末期肾病（ESRD）。虽然这两种病症之间的关联罕见，但鉴于患者的肾脏及生命预后，不应被忽视。
