Rheology of paraproteinaemias and the plasma hyperviscosity syndrome.

The proper understanding of the causes, pathophysiology, diagnosis and management of the plasma hyperviscosity syndrome is based on good knowledge of malignant paraproteinaemias, properties of immunoglobulins, rheology of blood in the microcirculation, and modern plasma separation techniques. This multifaceted syndrome complicates less than ten per cent of IgA and IgG myelomas, and up to one-third of Waldenström's macroglobulinaemias. A few cases of HVS have also been reported in association with polyclonal hypergammaglobulinaemias. Excessive paraproteinaemia may cause the plasma HVS, especially when paraproteins are extraordinarily large, asymmetrical or cryosensitive, or if they aggregate into hyperviscous macroaggregates. The resultant severe microcirculatory impairment is mainly due to the combined effects of plasma hyperviscosity, significant plasma volume expansion and intense red cell aggregation. The individually variable general symptoms, bleeding tendency, ocular, neurological, cardiovascular, and renal manifestations and laboratory parameters of the HVS are summarized briefly. The majority of patients present hyperviscosity manifestations when the plasma viscosity exceeds 5-6 mPa.s. Plasmapheresis or plasma exchange have established themselves as efficient and safe modes of therapy of hyperviscosity and hypervolaemia. The therapeutic guidelines for the plasma HVS are briefly discussed with regard to recent experience with developing plasma separation techniques. Diagnostic and therapeutic advances combined with increasing haemorheological knowledge have greatly improved the proper management of this potentially lethal complication of paraproteinaemias.