How I treat monoclonal gammopathy of renal significance (MGRS)

Author:

Fermand Jean-Paul1,Bridoux Frank2,Kyle Robert A.3,Kastritis Efstathios4,Weiss Brendan M.5,Cook Mark A.6,Drayson Mark T.7,Dispenzieri Angela3,Leung Nelson389

Affiliation:

1. University Hospital Center St. Louis, Paris, Ile de France, France;

2. Department of Nephrology and Transplantation, University Hospital Center Poitiers, Poitiers, France,

3. Division of Hematology, Mayo Clinic, Rochester, MN;

4. Department of Clinical Therapeutics, Alexandra Hospital, University of Athens, School of Medicine, Athens, Greece;

5. Abramson Cancer Center of the University of Pennsylvania, Philadelphia, PA;

6. Department of Laboratory Medicine and Pathology, and Departments of Hematology, and

7. Department of Immunity and Infection, University of Birmingham, Birmingham, UK;

8. Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN; and

9. Centre National de Référence Amylose AL, Poitiers, France

Abstract

Abstract Recently, the term monoclonal gammopathy of renal significance (MGRS) was introduced to distinguish monoclonal gammopathies that result in the development of kidney disease from those that are benign. By definition, patients with MGRS have B-cell clones that do not meet the definition of multiple myeloma or lymphoma. Nevertheless, these clones produce monoclonal proteins that are capable of injuring the kidney resulting in permanent damage. Except for immunoglobulin light chain amyloidosis with heart involvement in which death can be rapid, treatment of MGRS is often indicated more to preserve kidney function and prevent recurrence after kidney transplantation rather than the prolongation of life. Clinical trials are rare for MGRS-related kidney diseases, except in immunoglobulin light chain amyloidosis. Treatment recommendations are therefore based on the clinical data obtained from treatment of the clonal disorder in its malignant state. The establishment of these treatment recommendations is important until data can be obtained by clinical trials of MGRS-related kidney diseases.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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