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Clin J Am Soc Nephrol. 2016 Dec 7;11(12):2280-2287. Epub 2016 Aug 15.

Paraprotein-Related Kidney Disease: Diagnosing and Treating Monoclonal Gammopathy of Renal Significance.

Author information

1
Division of Nephrology, University of Virginia Health System, Charlottesville, Virginia.
2
Kidney Diseases Branch, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland.
3
Department of Nephrology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
4
Renal Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
5
Department of Medicine, Weill Cornell Medical College, New York, New York; and.
6
Divisions of Nephrology and Hypertension and leung.nelson@mayo.edu.
7
Hematology, Mayo Clinic, Rochester, Minnesota.

Abstract

Paraprotein-related kidney disease represents a complex group of diseases caused by an abnormal paraprotein secreted by a clone of B cells. The disease manifestations range from tubulopathies, such as the Fanconi syndrome, to a spectrum of glomerular diseases that can present with varying degrees of proteinuria and renal dysfunction. Diagnosis of these diseases can be challenging because of the wide range of manifestations as well as the relatively common finding of a serum paraprotein, especially in elderly patients. Thus, renal biopsy along with detailed hematologic workup is essential to link the presence of the paraprotein to the associated renal disease. Recent advances in treatment with more effective and targeted chemotherapies, as well as stem cell transplantation, have improved the renal and overall prognosis for many of these disorders.

KEYWORDS:

Aged; B-Lymphocytes; Biopsy; Fanconi Syndrome; Humans; Kidney Diseases; Paraproteins; Prognosis; Stem Cell Transplantation; kidney; kidney disease; monoclonal gammopathy; multiple myeloma; paraproteinemia; proteinuria

PMID:
27526705
PMCID:
PMC5142062
DOI:
10.2215/CJN.02920316
[Indexed for MEDLINE]
Free PMC Article

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