Wang L Y, Li X, Shen M, Chen S L, Huang Z X
Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100043, China.
Zhonghua Nei Ke Za Zhi. 2020 Feb 1;59(2):161-164. doi: 10.3760/cma.j.issn.0578-1426.2020.02.014.
A 49-year-old woman was admitted to hospital with intermittent dizziness and fatigue for 7 years. The symptoms were aggravated and accompanied by bone pain for more than 4 months. She was referred to our hospital. Laboratory tests and imaging findings suggested that acquired Fanconi Syndrome (FS) was associated with smoldering multiple myeloma (MM). Renal biopsy and electron microscopy confirmed the diagnosis of proximal light chain tubular disease (LCPT). LCPT causes proximal tubular dysfunction, which is characterized by the cytoplasmic crystal deposition usually kappa monoclonal light chain in the proximal tubule. MM with FS and LCPT is less common in clinical practice because it is difficult to diagnose. This is a typical case focusing on the differential diagnosis of monoclonal gammopathy of renal significance(MGRS) such as LCPT and plasma cells diseases.
一名49岁女性因间歇性头晕和乏力7年入院。症状加重并伴有骨痛4个多月。她被转诊至我院。实验室检查和影像学检查结果提示,获得性范科尼综合征(FS)与冒烟型多发性骨髓瘤(MM)相关。肾活检和电子显微镜检查确诊为近端轻链肾小管病(LCPT)。LCPT导致近端肾小管功能障碍,其特征是近端小管中通常为κ单克隆轻链的细胞质晶体沉积。伴有FS和LCPT的MM在临床实践中较少见,因为难以诊断。这是一个典型病例,重点关注具有肾意义的单克隆丙种球蛋白病(MGRS)如LCPT和浆细胞疾病的鉴别诊断。