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Clinical course and management of monoclonal gammopathy of undetermined significance

Author
S Vincent Rajkumar, MD
Section Editor
Robert A Kyle, MD
Deputy Editor
Rebecca F Connor, MD

INTRODUCTION

Monoclonal gammopathy of undetermined significance (MGUS) is an asymptomatic premalignant clonal plasma cell or lymphoplasmacytic proliferative disorder.

MGUS occurs in over 3 percent of the general population over the age of 50 and is typically detected as an incidental finding when patients undergo a protein electrophoresis as part of an evaluation for a wide variety of clinical symptoms and disorders (eg, peripheral neuropathy, vasculitis, hemolytic anemia, skin rashes, hypercalcemia, or elevated erythrocyte sedimentation rate).

The clinical course and management of patients with MGUS will be discussed here [1-4]. The diagnosis of MGUS, recognition of serum or urinary monoclonal proteins and the clinical features, laboratory manifestations, and diagnosis of multiple myeloma and other plasma cell dyscrasias are presented separately. (See "Diagnosis of monoclonal gammopathy of undetermined significance" and "Recognition of monoclonal proteins" and "Clinical features, laboratory manifestations, and diagnosis of multiple myeloma" and "Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia" and "Pathogenesis of immunoglobulin light chain (AL) amyloidosis and light and heavy chain deposition diseases".)

CLINICAL COURSE

Disease progression — There are three distinct clinical types of monoclonal gammopathy of undetermined significance (MGUS), each with a small risk of progressing through a unique intermediate (more advanced) premalignant stage and then to a malignant plasma cell dyscrasia or lymphoproliferative disorder [5-10]. Conversely, two large longitudinal studies have suggested that virtually all patients diagnosed with multiple myeloma (MM) had a preceding MGUS, with 75 percent having a detectible M-protein ≥8 years prior to the diagnosis of MM [8,9]. Likewise, another large study showed that all patients with immunoglobulin light chain (AL) amyloidosis had a preceding MGUS, with evidence of a monoclonal gammopathy being present in 80 percent at least four years prior and in 42 percent more than 11 years prior to the diagnosis of amyloidosis [11].

Non-IgM MGUS (IgG, IgA, or IgD MGUS) is the most common subtype of MGUS [12]. A minority of cases will progress to the more advanced premalignant stage smoldering (asymptomatic) MM and to symptomatic MM. Less frequently, these patients progress to AL amyloidosis, light chain deposition disease, or another lymphoproliferative disorder.

             

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Literature review current through: Nov 2016. | This topic last updated: Wed Jun 01 00:00:00 GMT 2016.
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