Abstract：
Monoclonal Ig crystalline nephropathies are rare lesions resulting from precipitation of monoclonal Igs in the kidney as crystalline inclusions. They can be categorized into lesions with predominant intracellular crystals (light chain [LC] proximal tubulopathy, LC crystal-storing histiocytosis, and LC crystalline podocytopathy) and lesions with predominant extracellular crystals (crystalglobulin-induced nephropathy and crystalline variant of LC cast nephropathy). The majority of these lesions are associated with low tumor burden lymphoproliferative disorders, with the exception of crystalline variant of LC cast nephropathy. Extrarenal involvement (e.g., skin and cornea) is frequent. Kidney biopsy is the cornerstone for diagnosis, which often requires electron microscopy and antigen retrieval. A thorough hematologic workup and evaluation of extrarenal involvement is mandatory for management. Treatment of these lesions is with clone-directed therapy, with the goal of achieving hematologic very good partial response or complete response, which preserves or improves kidney function. In vitro and in vivo studies, animal models, and novel sequencing techniques have been invaluable tools to understand the pathogenesis of LC proximal tubulopathy and can be used to increase our limited knowledge of the pathogenesis of the other monoclonal Ig crystalline nephropathies. This review provides an update on the pathology, renal and hematologic characteristics, extrarenal manifestations, prognosis, treatment, and pathogenesis of monoclonal Ig crystalline nephropathies.
摘要：
单克隆免疫球蛋白（MIg）结晶肾病是罕见的病变，是由于肾脏中MIgs作为结晶内含物沉淀而引起的。它们可以分类为具有主要细胞内晶体的病变（轻链[LC]近端肾小管病，LC晶体储存组织细胞增生症，LC结晶性足细胞病]和具有主要细胞外晶体的病变（晶体球蛋白诱导的肾病，LC铸型肾病的结晶变体)。这些病变中的大多数与低肿瘤负荷的淋巴增生性疾病有关，除了LC铸型肾病的结晶变体。肾外受累（例如，皮肤,角膜)是常见的。肾活检是诊断的基石,这通常需要电子显微镜和抗原检索。必须进行彻底的血液学检查和评估肾外受累。这些病变的治疗是克隆导向治疗，目的是达到血液学非常好的部分反应或完全反应，从而保持或改善肾功能。体外和体内研究，动物模型,和新的测序技术已成为了解LC近端肾小管病发病机制的宝贵工具，并可用于增加我们对其他Mig结晶性肾病的发病机理的有限知识。这篇综述将提供病理学的最新情况，肾脏和血液学特征，肾外表现,预后,治疗,MIg结晶肾病的发病机制。
