Pathophysiology and management of monoclonal gammopathy of renal significance

Author:

Jain Ankur1,Haynes Richard23,Kothari Jaimal45,Khera Akhil4,Soares Maria6,Ramasamy Karthik457ORCID

Affiliation:

1. Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India;

2. Oxford Kidney Unit, Oxford University Hospitals, NHS Foundation Trust, Oxford, United Kingdom;

3. MRC Population Health Research Unit, Clinical Trial Service Unit and Epidemiological Studies Unit, Nuffield Department of Population Health, Headington, Oxford, United Kingdom;

4. Department of Haematology, Oxford University Hospitals, NHS Foundation Trust, Oxford, United Kingdom;

5. NIHR Biomedical Research Centres Blood Theme, Oxford, United Kingdom;

6. Department of Cellular Pathology, John Radcliffe Hospital, Oxford University Hospitals, NHS Foundation Trust, Oxford, United Kingdom; and

7. Oxford Myeloma Centre for Translational Research, Oxford, United Kingdom

Abstract

AbstractRecent years have witnessed a rapid growth in our understanding of the pathogenic property of monoclonal proteins. It is evident that some of these small monoclonal proteins are capable of inducing end-organ damage as a result of their intrinsic physicochemical properties. Hence, an umbrella term, monoclonal gammopathy of clinical significance (MGCS), has been coined to include myriad conditions attributed to these pathogenic proteins. Because kidneys are the most commonly affected organ (but skin, peripheral nerves, and heart can also be involved), we discuss MGRS exclusively in this review. Mechanisms of renal damage may involve direct or indirect effects. Renal biopsy is mandatory and demonstration of monoclonal immunoglobulin in kidney, along with the corresponding immunoglobulin in serum or urine, is key to establish the diagnosis. Pitfalls exist at each diagnostic step, and a high degree of clinical suspicion is required to diagnose MGRS. Recognition of MGRS by hematologists and nephrologists is important, because timely clone-directed therapy improves renal outcomes. Autologous stem cell transplant may benefit selected patients.

Publisher

American Society of Hematology

Subject

Hematology

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