Improvement of Light Chain Proximal Tubulopathy without Crystals in IgG?-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone
light chain proximal tubulopathy without crystals;
monoclonal gammopathy of renal significance;
monoclonal gammopathy of undetermined significance;
paraprotein-related kidney disease;
ADULT FANCONI-SYNDROME;
D O I:
10.2169/internalmedicine.1835-23
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
A 70 -year -old woman with acute kidney injury, a high serum Creatinine (Cr) level (3.91 mg/dL), and proteinuria (protein/Cr ratio 1.59 g/gCr) was admitted. Serum IgG ? -type and urinary ? -type M proteins were observed. A bone marrow examination indicated monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy showed distended proximal tubular cells, and immunofluorescence identified tissue positive for proximal tubular cell ? light chains. Electron microscopy identified fibril-like structures in the lysosomes. The patient was diagnosed with light chain proximal tubulopathy without crystals in IgG ? -type MGUS and treated with bortezomib and dexamethasone therapy, which improved her renal function.
机构:
Kameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, JapanKameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, Japan
Nishida, Yuki
Iwama, Kan-ichi
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机构:
Kameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, JapanKameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, Japan
Iwama, Kan-ichi
Yamakura, Masayuki
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机构:
Kameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, JapanKameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, Japan
Yamakura, Masayuki
Takeuchi, Masami
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Kameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, JapanKameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, Japan
Takeuchi, Masami
Matsue, Kosei
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机构:
Kameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, JapanKameda Gen Hosp, Dept Med, Div Hematol Oncol, Kamogawa, Chiba 2968602, Japan