Case 1. Patient 1 is a 65-year-old plumber who presents with fatigue, headache, blurred vision, and intermittent nosebleeds for the past 3 months. Examination demonstrates mild generalized lymphadenopathy and a palpable spleen tip. The fundi show marked retinal vein engorgement with "sausaging." Laboratory tests disclose mild anemia and a large monoclonal spike on serum protein electrophoresis. How should he be managed?Elevated blood, plasma, or serum viscosity occurs in a number of hematologic disorders. Hyperviscosity syndrome (HVS) is a clinical feature in 10% to 30% of patients with Waldenström macroglobulinemia (WM), sometimes as its presenting manifestation. 1 HVS also accompanies other conditions, such as multiple myeloma, rheumatoid disease, polycythemia, sickle cell disease, leukemia, and spherocytosis. 2 The latter 4 cellular causes of HVS are beyond the scope of this discussion. The purpose of this paper is to discuss the characteristic features of HVS secondary to elevated plasma or serum viscosity and to evaluate evidence supporting various diagnostic and treatment approaches using the Grade system (Table 1). 3,4 In searching the Medline database on hyperviscosity syndrome, 594 references were listed back to 1965. A limited number of older publications were also used. Only English-language articles were considered. Observational studies, systemic reviews, or case studies were included. No randomized trials on management of HVS were identified.
Methods to measure viscosityViscosity refers to resistance to flow or stickiness, from the Latin word "viscum alba" for mistletoe. 5,6 (Mistletoe berries were once used to make a viscous glue.) Viscosity is classically measured in one of 2 ways: by determining the rate of fluid flow as a result of applying a predefined force or by measuring the amount of force required to achieve a predefined rate of fluid flow.Although HVS is caused by hyperviscous blood, clinical laboratories generally only measure the serum or plasma component. Serum and plasma display Newtonian properties in that viscosity is independent of pressure drop or velocity gradient. Consequently, the method of measurement will not dramatically affect the test result. By contrast, whole blood viscosity is complex because of the suspension of red cells in plasma, resulting in non-Newtonian behavior. Different methods of measurement can yield various results.In WM, serum or plasma viscosity measurements reflect the amount and properties of the IgM paraprotein. Monoclonal IgMs display a wide range of intrinsic viscosity values (0.106-0.162 dL/ g), each protein having individual properties. 7,8 However, relative viscosity values are highly reproducible for any individual protein.IgM is a star-shaped pentamer with a molecular size of 925 kDa (IgG is 150 kDa and albumin is 65 kDa). Thus, it is not surprising that this giant IgM molecule, which is 80% intravascular, exerts profound effects on blood flow and cells, especially when present in the high concentrations found in WM patients.The methods used fo...