摘要
患者女,49岁,临床主要表现为骨痛和蛋白尿。因间歇性头晕、疲倦7年,症状加重并伴骨痛4个月余入院。患者在当地医院被诊断为贫血,并疑诊多发性骨髓瘤(MM)。实验室和影像学检查结果提示为获得性范科尼综合征(FS)伴有冒烟型多发性骨髓瘤(SMM),经肾活检、电子显微镜检查和反复入院检查证实为轻型近端肾小管病(LCPT)。LCPT常引起近端肾小管功能障碍,其特征是近端小管中结晶,多为kappa单克隆轻链的胞质内结晶状沉积。具有FS和LCPT特征的MM患者在临床上较少见,且难以准确诊断。希望通过此病例的分析,使临床医生关注LCPT等类型的有肾脏意义的单克隆球蛋白病(MGRS),以及与浆细胞病(如MM)的鉴别诊断。
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.
作者
王林月
李新
申曼
陈世伦
黄仲夏
Wang Linyue;Li Xin;Shen Man;Chen Shilun;Huang Zhongxia(Department of Hematology,Multiple Myeloma Medical Center of Beijing,Beijing Chaoyang Hospital,Capital Medical University,Beijing 100043,China)
出处
《中华内科杂志》
CAS
CSCD
北大核心
2020年第2期161-164,共4页
Chinese Journal of Internal Medicine
基金
北京市石景山区医学重点扶持专科建设项目(20170006)
北京市卫生和计划生育委员会科技成果和适宜技术推广项目(2018-TG-07)。
