![]() ![]() Plasmapheresis, first carried out manually for macroglobulinemia in the late 1950s, was demonstrated to reverse retinopathy and other clinical manifestations in most patients with HVS. 12, 14, 16-18 Protein coating also contributes to a platelet functional defect that further accentuates the bleeding tendency. 1, 12, 14-18, 20-22 In addition to raising plasma viscosity, macroglobulin coats red cells, leading to the characteristic stacking appearance (rouleaux) on peripheral blood smear in WM patients. Prompt diagnosis of HVS from the eye examination enables the institution of appropriate therapy (ie, plasmapheresis). Hemorrhages, exudates, microaneurysms, papilledema, and an appearance indistinguishable from central retinal vein occlusion may be seen in later stages. 1, 12, 14-18 The HVS can be diagnosed from the physical examination by the funduscopic finding of marked retinal venous engorgement resembling hot dogs on a string (ie, “sausaging” 12, 14-18, 20 Figure 1). 7, 8 However, viscosity values correlate closely with signs and symptoms in the same patient (“symptomatic threshold”). Such variation is the result, in part, of the previously mentioned wide range of intrinsic viscosity values noted in monoclonal macroglobulins. Viscosity levels in HVS vary significantly between patients. ![]()
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