Color Doppler ultrasound investigation of the therapeutic effect of plasmapheresis on a lymphoplasmocytoid lymphoma (immunocytoma): a case of central retinal artery occlusion.

The lymphoplasmocytoid immunocytoma (Waldenström's disease) is a non-Hodgkin's lymphoma of low malignancy. The disease is defined by the production of monoclonal IgM-globulins in the bone marrow. These macroglobulins affect the aggregation of thrombocytes and bind the coagulation factors, which leads not only to hemorrhagical diathesis, but also to hyperviscosity syndrome. We present the rare case of a 79-year-old woman suffering from an immunocytoma with sudden painless loss of vision in her right eye. Ophthalmoscopic examination revealed an occlusion of the central retinal artery. The immediately performed blood examination showed the following results: IgM serum level was 6610 mg/dl (normal range: 60-370 mg/ dl), kappa-paraproteinemia showed values of 702 mg/dl (normal range: 200-440 mg/dl), and the plasma viscosity was 3.4 mPa/s (normal range: 1.5-1.72 mPa/s). Plasmapheresis was promptly initiated. Subsequently, the highly pathological serum levels were markedly lower and sight improved from complete amaurosis to qualitative vision. Blood-flow velocity and vascular resistance parameters (resistive index) in all nutritious vessels [ophthalmic artery (OA), posterior ciliar arteries (PCA) and central retinal artery (CRA)] were recorded before and after each of the six cycles of plasmapheresis, using color Doppler velocity (CDV). It was proven that even after the first plasmapheresis, circulation in the central retinal artery was restored. After each further cycle of plasmapheresis, an improvement in blood-flow velocity in OA, PCA, and CRA was recorded by CDV. CDV verifies variations in circulation of the eye vessels and gives an objective assessment of the influence of therapy.