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Identification of gammopathy as IgM-predominant
11%
2/18
Identification of gammopathy as IgG-predominant
6%
1/18
Identification of gammopathy as IgA-predominant
The presence of engorged retinal veins
39%
7/18
Splenomegaly
33%
6/18
Select Answer to see Preferred Response
This patient's clinical presentation is consistent with a monoclonal gammopathy. Splenomegaly is found in both Waldenstrom's macroglobulinemia and multiple myeloma. The differential diagnosis of an M-spike on SPEP includes multiple myeloma, smoldering myeloma, monoclonal gammopathy of unknown significance (MGUS), and Waldenstrom's macroglobulinemia (WM). WM should be considered in the differential of patients presenting with systemic symptoms and a monoclonal gammopathy. Evidence of increased blood viscosity, such as tortuous retinal veins, supports a diagnosis of WM rather than multiple myeloma or MGUS. In addition, while multiple myeloma typically presents with a monoclonal IgA or IgG population, WM is associated with monoclonal IgM. George and Sadovsky review the recognition and management of multiple myeloma. They note that the incidence of a monoclonal gammopathy is near 8% in elderly populations, and patients with MGUS must be monitored closely for development of multiply myeloma. In addition to MGUS and WM, the differential diagnosis should include heavy-chain disease and a solitary plasmacytoma, however these conditions are less common. Kyle et al. perform a retrospective cohort study to determine the long-term prognosis of patients with MGUS. They find that the risk of progression to multiple myeloma or another related disorder is approximately 1% per year in their study population. In addition, they note that the initial concentration of monoclonal antibody was a positive predictor of progression to multiple myeloma over the following 20 years. Illustration A shows a normal SPEP at the top, and an SPEP with a monoclonal gammopathy at the bottom. Illustration B shows tortuous retinal veins resulting from blood hyperviscosity in a patient with WM. Illustration C shows a bone marrow aspirate from a patient with WM; note the proliferation of immature lymphocytes. Incorrect Answers: Answer 1: IgM-predominance supports a diagnosis of WM. Answers 2-3: IgG or IgA predominance supports a diagnosis of multiple myeloma. Answer 4: Engorged retinal veins is evidence of blood hyperviscosity, which is associated with WM.
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