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Kidney Int. 2015 Apr;87(4):698-711. doi: 10.1038/ki.2014.408. Epub 2015 Jan 21.

Diagnosis of monoclonal gammopathy of renal significance.

Author information

1
Department of Nephrology and Transplantation, Centre Hospitalier Universitaire de Poitiers and Centre national de référence maladies rares: amylose AL et autres maladies à depots d'immunoglobulines monoclonales; Université de Poitiers, Poitiers, France.
2
1] Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA [2] Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
3
Department of Medicine, University of Otago, Wellington, New Zealand.
4
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
5
Department of Hematology and Immunology, Univerisity Hospital St Louis, Paris, France.
6
Department of Pathology, Loyola University Medical Center, Maywood, Illinois, USA.
7
Department of Pathology, Louisiana State University Health Sciences Center, Shreveport, Louisiana, USA.
8
Department of Clinical Therapeutics, Alexandra Hospital, University of Athens School of Medicine, Athens, Greece.
9
Department of Molecular Medicine, University of Pavia, Pavia, Italy.
10
Hématologie Clinique, Hôpital Purpan, Toulouse, France.
11
Division of Nephrology and Hypertension, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.
12
Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Abstract

Monoclonal gammopathy of renal significance (MGRS) regroups all renal disorders caused by a monoclonal immunoglobulin (MIg) secreted by a nonmalignant B-cell clone. By definition, patients with MGRS do not meet the criteria for overt multiple myeloma/B-cell proliferation, and the hematologic disorder is generally consistent with monoclonal gammopathy of undetermined significance (MGUS). However, MGRS is associated with high morbidity due to the severity of renal and sometimes systemic lesions induced by the MIg. Early recognition is crucial, as suppression of MIg secretion by chemotherapy often improves outcomes. The spectrum of renal diseases in MGRS is wide, including old entities such as AL amyloidosis and newly described lesions, particularly proliferative glomerulonephritis with monoclonal Ig deposits and C3 glomerulopathy with monoclonal gammopathy. Kidney biopsy is indicated in most cases to determine the exact lesion associated with MGRS and evaluate its severity. Diagnosis requires integration of morphologic alterations by light microscopy, immunofluorescence (IF), electron microscopy, and in some cases by IF staining for Ig isotypes, immunoelectron microscopy, and proteomic analysis. Complete hematologic workup with serum and urine protein electrophoresis, immunofixation, and serum-free light-chain assay is required. This review addresses the pathologic and clinical features of MGRS lesions, indications of renal biopsy, and a proposed algorithm for the hematologic workup.

PMID:
25607108
DOI:
10.1038/ki.2014.408
[Indexed for MEDLINE]

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