![]() The term “smoldering multiple myeloma” was created by Kyle and Greipp 1 in 1980 to classify a group of patients with evidence of a clear monoclonal gammopathy of reasonably high burden but for whom “treatment should be withheld” given the lack of myeloma-defining events. ![]() He is diagnosed with high-risk smoldering multiple myeloma based on an M-spike greater than 2 g/dL and a kappa/lambda ratio greater than 20. He has no other myeloma-defining events based on imaging and blood test results. ![]() An evaluation of the patient’s neuropathy reveals findings suggestive of diabetic neuropathy. In the first of three scenarios, the patient is found to have an immunoglobulin (Ig) G kappa monoclonal gammopathy measuring 2.2 g/dL, serum free light chains reveal a kappa/lambda ratio of 24, and a bone marrow biopsy reveals 15% kappa-restricted plasmacytosis. 1 ), 4 – 8 making the approach to the diagnosis and management of these conditions all the more challenging. 3 What this means for the practicing clinician is that any given monoclonal gammopathy can have many different fates ( Fig. 2 In a recent, prospective, population-wide screening study of the adult Icelandic population (older than age 40), MGUS was identified in 4.9% of the population at baseline, with the prevalence increasing with age 1% of patients have thus far been diagnosed with a lymphoproliferative disorder, including multiple myeloma, light-chain (AL) amyloidosis, chronic lymphocytic leukemia, and Waldenström macroglobulinemia (WM). It is estimated that the risk of progression from MGUS to multiple myeloma is approximately 1% per year. ![]() 1 The majority of MGUS cases arise from a malignant plasma cell clone in the bone marrow that does not reach the diagnostic threshold of multiple myeloma. Until recently, the incidence of monoclonal gammopathy of undetermined significance (MGUS) was based on studies from Olmsted County, Minnesota, and from Scandinavian health records, indicating an incidence of approximately 1%. Waldenström macroglobulinemia is an important cause of IgM monoclonal gammopathy, which can be associated with a wide variety of clinical features recently developed targeted therapies can achieve durable responses.Ī monoclonal gammopathy is a laboratory-based finding that is being increasingly recognized in the aging population. Light-chain amyloidosis incidence is paramount given that delayed diagnosis hampers treatment success and may result in irreversible organ damage.Īccurate typing of amyloidosis is important in distinguishing between light-chain, transthyretin, and other forms of amyloidosis. Smoldering multiple myeloma is a clinical entity that does not require immediate treatment, but selected patients with high-risk disease require more frequent follow-up. Monoclonal gammopathies represent a broad differential diagnosis and require a thorough evaluation for clinical significance. In this review, we focus on treatment strategies for patients with monoclonal gammopathies that are not multiple myeloma, including smoldering multiple myeloma, light-chain amyloidosis, and Waldenström macroglobulinemia. Recognition has become all the more important as we understand how to triage the 4% to 9% of patients with monoclonal gammopathies depending on age, with the goal of limiting overdiagnosis and misdiagnosis. Early recognition of monoclonal gammopathies along with a careful workup are essential in determining the next steps in the care of a given patient. This change has created a challenge for patients and clinicians, as a monoclonal gammopathy may be a harbinger not of multiple myeloma but of other lymphoproliferative disorders such as light-chain amyloidosis and Waldenström macroglobulinemia. Once accepted as a possible precursor condition to multiple myeloma, monoclonal gammopathies as an entity are now associated with many renal, neurologic, and dermatologic disorders of clinical significance. Our knowledge of monoclonal gammopathies is continuously evolving.
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