我们在此报告一例合并的结晶轻链肾小管病,足细胞病,组织细胞增生症,肾意义单克隆丙种球蛋白病(MGRS)患者的铸型肾病。一名66岁的肾功能受损的女性被转诊到我们部门。尽管进行了静脉液体复苏,肾功能逐渐恶化;因此,进行了肾活检.肾活检显示轻链近端肾小管病变(LCPT)伴晶体,轻链晶体足细胞病(LCCP),储存晶体的组织细胞增多症(CSH),和轻链铸造肾病(LCCN)。值得注意的是,通过电子显微镜诊断LCCP和CSH。血清和尿液免疫电泳(IEP)显示存在单克隆Bence-Jones蛋白和游离κ轻链。骨髓抽吸显示浆细胞增殖<10%。因此,我们曾遇到一例罕见病例,其中多种肾脏病变合并MGRS.大部分LCPT,LCCP,CSH病例显示单克隆IgGκ,而我们的病例显示Bence-Jones蛋白κ。
We herein report a case of a combined crystalline light chain tubulopathy, podocytopathy, histiocytosis, and cast nephropathy in a patient with monoclonal gammopathy of renal significance (MGRS). A 66-year-old female with impaired renal function was referred to our department. Despite intravenous fluid resuscitation, the kidney function worsened progressively; thus, a kidney biopsy was performed. The kidney biopsy revealed light chain proximal tubulopathy (LCPT) with crystals, light chain crystal podocytopathy (LCCP), crystal-storing histiocytosis (CSH), and light chain cast nephropathy (LCCN). Of note, LCCP and CSH were diagnosed via electron microscopy. Serum and urine immunoelectrophoresis (IEP) revealed the presence of monoclonal Bence-Jones protein and free κ light chains. Bone marrow aspiration showed < 10% plasma cell proliferation. Thus, we had encountered a rare case in which a variety of kidney lesions were combined with MGRS. Most of the LCPT, LCCP, and CSH cases show monoclonal IgG κ, while our case showed Bence-Jones protein κ.