Improvement of Light Chain Proximal Tubulopathy without Crystals in IgG?-type Monoclonal Gammopathy of Undetermined Significance Using Bortezomib and Dexamethasone

被引:2
|
作者
Tsuyuki, Tomohisa [1 ]
Uramatsu, Tadashi [2 ]
Shimizu, Masatoshi [1 ]
Ishi, Takuma [1 ]
Tsuji, Kiyokazu [2 ]
Nakashima, Jun [3 ]
Katafuchi, Eisuke [4 ]
Nakayama, Toshiyuki [4 ]
Uesugi, Noriko [5 ]
Muta, Kumiko [1 ]
Nishino, Tomoya [1 ]
机构
[1] Nagasaki Univ Hosp, Dept Nephrol, Nagasaki, Japan
[2] JCHO Isahaya Gen Hosp, Dept Neurol, Isahaya, Japan
[3] JCHO Isahaya Gen Hosp, Dept Hematol, Isahaya, Japan
[4] Univ Occupat & Environm Hlth Sch, Dept Pathol, Kitakyushu, Japan
[5] Fukuoka Univ Hosp, Dept Pathol, Fukuoka, Japan
关键词
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.
引用
收藏
页码:693 / 698
页数:6
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