Hyperviscosity syndrome occurs in fewer than 5% of IgG and in 5-10% of IgA myeloma patients.98'99 The type of monoclonal immunoglobulin and the plasma concentration are the main determinants for the development of hyperviscosity. In one study of IgG myeloma, hyperviscosity occurred in 4.2% of 238 patients, and in 22% of 46 patients with serum IgG level above 5.0 g/dL.98 The IgG3 subtype, with a tendency for aggregation, and unusually asymmetrical IgG molecules with a high axial ratio, pose higher risks.100101 Similarly, myeloma patients with polymeric IgA are much more likely to develop hyperviscosity syndrome than those with the monomeric form.99102 Hyperviscosity has also been reported in unusual cases of light-chain myeloma.103
The circulatory disturbances resulting from hyper-viscosity lead to various clinical manifestations. Headache, blurred vision, reduced visual acuity, and drowsiness are common. Occasionally, patients may present with dementia or psychosis.104 Progressive, severe CNS dysfunction results in obtundation, vertigo, seizure, gait ataxia, and coma. Dyspnea may precede overt congestive heart failure. Bleeding occurs most commonly as epistaxis, ecchymosis, and sometimes GI
bleeding. Fundoscopic examination, revealing characteristic retinal flame-shaped hemorrhages, engorged, tortuous, segmented retinal veins ("box-caring"), and papilledema, should be performed initially and repeatedly during the course of treatment.
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