Irreversible visual loss in Waldenström's macroglobulinaemia.

A patient with Waldenström's macroglobulinaemia presented with visual reduction in both eyes. The funduscopic and angiographic demonstrations of venous engorgement ('string of sausages'), retinal haemorrhages at all levels, retinal and disc oedema, and serous detachment of the maculas were consistent with this diagnosis. The cryoprecipitation of the immunoglobulin at a temperature slightly below body temperature precluded routine blood studies and plasmapheresis. Plasmapheresis was ultimately performed without difficulty with the patient and equipment at 88 degrees F (31 degrees C). Despite marked improvement in the funduscopic and angiographic appearance of the retina, perifoveal capillary nonperfusion and serous elevation of the macula persisted. Even when the maculas flattened in both eyes, no visual recovery occurred. Early diagnosis, even on a clinical basis when laboratory studies cannot be performed, and early plasmapheresis to reduce serum viscosity are warranted to prevent intravascular occlusion in the perifoveal capillary bed, deposition of immunoglobulin in the retina, and transudation in the subretinal space.