2014年5月
Gamma 1-heavy chain deposition disease accompanied by IgG kappa in serum, urine, and bone marrow
CEN Case Reports
- ,
- ,
- ,
- ,
- ,
- ,
- ,
- 巻
- 3
- 号
- 1
- 開始ページ
- 44
- 終了ページ
- 48
- 記述言語
- 英語
- 掲載種別
- 研究論文(学術雑誌)
- DOI
- 10.1007/s13730-013-0083-0
- 出版者・発行元
- Springer Science and Business Media LLC
A 32-year-old Japanese woman presented with hypertension, nephrotic syndrome, microhematuria, and severe hypocomplementemia. Her serum creatinine concentration increased from 1.46 mg/dL (129.0 μmol/L) to 3.46 mg/dL (305.8 μmol/L) over 1 month. Renal biopsy revealed Congo red-negative nodular glomerulosclerosis accompanied by mesangial proliferation. There was extensive staining of immunoglobulin (Ig) G in the glomerular and tubular basement membranes and expanded mesangial regions. Staining was negative for IgA, IgM, and kappa and lambda light chains and positive for the gamma 1 IgG subclass. Staining for constant domains of the gamma heavy chains showed a deletion of the first constant domain (CH1). Electron microscopy revealed electron-dense deposits in the glomerular and tubular basement membranes and mesangium. These findings indicated gamma 1-heavy chain deposition disease (HCDD). Serum and urine immunoelectrophoresis revealed an IgG kappa monoclonal band, whereas bone marrow biopsy revealed monoclonal plasmacytosis with positive staining for kappa chains. HCDD associated with kappa light chain is extremely rare. We report the first case of HCDD with IgG kappa detected in the serum, urine, and bone marrow.
- リンク情報
-
- DOI
- https://doi.org/10.1007/s13730-013-0083-0
- PubMed
- https://www.ncbi.nlm.nih.gov/pubmed/28509245
- PubMed Central
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5411537
- URL
- http://link.springer.com/content/pdf/10.1007/s13730-013-0083-0.pdf
- URL
- http://link.springer.com/article/10.1007/s13730-013-0083-0/fulltext.html
- URL
- http://link.springer.com/content/pdf/10.1007/s13730-013-0083-0
- ID情報
-
- DOI : 10.1007/s13730-013-0083-0
- eISSN : 2192-4449
- PubMed ID : 28509245
- PubMed Central 記事ID : PMC5411537