Abstract
In 44 of 56 patients with malignant paraproteinaemia the relationship of serum viscosity, plasma volume (PV), estimated blood volume (BV) and clinical findings was investigated and in the remaining 12 patients one or more of these parameters was studied. There was a correlation between increased PV and serum viscosity (r= 0.66, P<0.001). The retinopathy characteristic of the hyperviscosity syndrome (HVS) was always associated with serum viscosity ≥3.8 and with hypervolaemia. The evidence that the retinal changes were related more closely to serum hyperviscosity than to hypervolaemia is twofold. First, there was a considerable overlap in the degree of BV expansion, but not serum hyperviscosity, in patients with and without retinopathy. Second, the retinopathy in a patient with Waldenström's macroglobulinaemia (WM) improved after plasma exchange with little change in BV. While bleeding was often associated with retinopathy seven patients with multiple myeloma (MM) had no retinopathy, viscosities ≥4.3 and BV in the same range as patients with no clinical signs. Haemorrhage in such patients may thus be unrelated to the property of the paraprotein responsible for hyperviscosity. Measurements of PV in a patient with WM having repeated plasma exchanges for bleeding suggested that hypervolaemia may contribute to haemorrhage in some patients. It is suggested that the term ‘HVS’ should be restricted to patients with retinopathy and that hypervolaemia should be considered a characteristic feature of this syndrome.