Abstract
SummaryMonoclonal gammopathy of undetermined significance (MGUS) is a premalignant hematological condition arising from B‑cells, characterized by the presence of monoclonal immunoglobulin production, also known as paraprotein. It is found in up to 3% of individuals over the age of 50 years. The overall progression rate is low at around 1% per year, with most patients progressing to multiple myeloma (MM). Other diseases that may arise from MGUS include non-Hodgkin lymphomas, Waldenström macroglobulinemia, amyloid light-chain (AL) amyloidosis, POEMS (polyneuropathy, organomegaly, endocrinopathy, M‑protein, skin changes), cryoglobulinemia, monoclonal gammopathy of renal significance (MGRS), and additional paraprotein-driven diseases. Every case of monoclonal gammopathy warrants careful investigation to rule out the presence of a malignant form. In recent years, paraprotein-associated conditions such as MGRS have been increasingly recognized. Accurate diagnosis and work-up of these cases require a multidisciplinary approach, and it is paramount to accurately distinguish them from true MGUS in order to prevent end-organ damage. Follow-up of MGUS should be lifelong; however, a risk-adapted approach involving the primary care setting is recommended.
Highlights
Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant B-cell neoplasia usually arising from plasma cells and less frequently from lymphoplasmacytic cells
Kidney dysfunction with proteinuria should trigger evaluation for monoclonal gammopathy of renal significance (MGRS) or kidney amyloid light-chain (AL) amyloidosis, while cardiac failure that is rapidly progressing without any prior history of cardiac dysfunction or hypertrophic left ventricular cardiomyopathy can be suggestive for cardiac AL amyloidosis [14]
The exact prevalence rate of monoclonal gammopathy of clinical significance (MGCS) is unknown, since it remains underdiagnosed as the connection between symptoms and the underlying paraproteinemia are often overlooked
Summary
Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant B-cell neoplasia usually arising from plasma cells and less frequently from lymphoplasmacytic cells It is defined as a serum monoclonal protein of less than 30 g/L, less than 10% of clonal plasma or lymphoplasmacytic cells in the bone marrow, and the absence of end-organ damage, myeloma defining events, or any constitutional symptoms [1]. While it is a rare occurrence in patients younger than 50 years, it is found in 1.7% of individuals aged between 50 and 59 years, with slightly more men being affected. This proportion increases to up to 7.5% in persons older than 85 years. The most common immunoglobulin isotypes are immunoglobulin G (IgG) and Kappa light chain [2]
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