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Nat Rev Nephrol. 2019 Jan;15(1):45-59. doi: 10.1038/s41581-018-0077-4.

The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research Group.

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Division of Nephrology, Hematology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Department of Nephrology, Centre Hospitalier Universitaire et Université de Poitiers, Poitiers, France; CNRS UMR7276, Limoges, France; and Centre de Référence Amylose AL et Autres Maladies par Dépôt d'Immunoglobulines Monoclonales, Poitiers, France.
Veterans Administration Medical Center, New Orleans, LA, USA and Tulane University Medical School, Tulane, LA, USA.
Centre for Haematology, Department of Medicine, Imperial College London and Imperial College Healthcare NHS Trust, Hammersmith Hospital, London, UK.
Department of Nephrology, Renal Medicine - University Hospitals Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, UK.
Department of Pathology, Renal Pathology Laboratory, Columbia University, College of Physicians and Surgeons, New York, NY, USA.
Division of Nephrology, Hematology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Department of Haematology and Immunology, University Hospital St Louis, Paris, France.
The Victorian and Tasmanian Amyloidosis Service, Department of Haematology, Monash Univerity Easter Health Clinical School, Melbourne, Victoria, Australia.
National Amyloidosis Centre, Centre for Amyloidosis and Acute Phase Proteins, Division of Medicine, Royal Free Campus, University College London, London, UK.
Department of Pathology and Translational Pathobiology, Louisiana State University Health Sciences Center, Shreveport, LA, USA.
Service d'Hématologie et de Thérapie Cellulaire, Centre de Référence des Amyloses Primitives et des Autres Maladies par Dépôts d'Immunoglobuline, CHU Limoges, Limoges, France.
Department of Clinical Therapeutics, School of Medicine National and Kapodistrian University of Athens Alexandra Hospital, Athens, Greece.
University Health Network, Princess Margaret Cancer Centre, Toronto, Canada.
Arkana Laboratories, Little Rock, AR, USA.
Wilhelminen Cancer Research Institute, Wilhelminenspital, Vienna, Austria.
Amyloidosis Research and Treatment Center, IRCCS Policlinico San Matteo, University of Pavia, Pavia, Italy.
Haematology Department, Princess Alexandra Hospital and School of Medicine, University of Queensland, Brisbane, Australia.
Department of Pathology, Loyola University Medical Center, Maywood, IL, USA.
Department of Pathology, Hôpital Maisonneuve-Rosemont, Université de Montreal, Montreal, Quebec, Canada.
Department of Medicine, University of Alabama at Birmingham and Department of Veterans Affairs Medical Center, Birmingham, AL, USA.
Cross Cancer Institute, University of Alberta, Edmonton, Alberta, Canada.
Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute, Atrium System, Charlotte, NC, USA.
Abramson Cancer Center, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.


The term monoclonal gammopathy of renal significance (MGRS) was introduced by the International Kidney and Monoclonal Gammopathy Research Group (IKMG) in 2012. The IKMG met in April 2017 to refine the definition of MGRS and to update the diagnostic criteria for MGRS-related diseases. Accordingly, in this Expert Consensus Document, the IKMG redefines MGRS as a clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin and does not meet previously defined haematological criteria for treatment of a specific malignancy. The diagnosis of MGRS-related disease is established by kidney biopsy and immunofluorescence studies to identify the monotypic immunoglobulin deposits (although these deposits are minimal in patients with either C3 glomerulopathy or thrombotic microangiopathy). Accordingly, the IKMG recommends a kidney biopsy in patients suspected of having MGRS to maximize the chance of correct diagnosis. Serum and urine protein electrophoresis and immunofixation, as well as analyses of serum free light chains, should also be performed to identify the monoclonal immunoglobulin, which helps to establish the diagnosis of MGRS and might also be useful for assessing responses to treatment. Finally, bone marrow aspiration and biopsy should be conducted to identify the lymphoproliferative clone. Flow cytometry can be helpful in identifying small clones. Additional genetic tests and fluorescent in situ hybridization studies are helpful for clonal identification and for generating treatment recommendations. Treatment of MGRS was not addressed at the 2017 IKMG meeting; consequently, this Expert Consensus Document does not include any recommendations for the treatment of patients with MGRS.


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