Primary macrocryogelglobulinemia. Remission with adrenal corticosteroid therapy

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Abstract
The patient had recurrent epistaxis and retinal and neurologic abnormalities attributable to an extraordinary increase in serum viscosity associated with a macroglobulin that formed a gel even at room and body temperatures. Dramatic clinical improvement and a decrease in serum viscosity but no reduction in the serum macroglobulin concentration occurred 3 weeks after initiation of adrenal corticosteroid therapy. The clinical remission was maintained for 13 months when therapy had to be discontinued due to infection. The serum hyperviscosity then recurred and the patient died shortly from hemorrhage.

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