Malignant exophthalmos associated with multiple myeloma

We report a patient with malignant exophthalmos associated with multiple myeloma which showed no evidence of direct orbital involvement of plasma cells. This exophthalmos had similarities with Graves' ophthalmopathy, but the patient had no detectable autoimmune thyroid diseases. Plasmapheresis was effective not only for the treatment of heart and renal failure due to the myeloma kidney but also for the malignant exophthalmos. As the serum monoclonal IgG level was decreased by plasmapheresis, the improvement of proptosis, visual acuity, and hypertrophy of the extraocular muscle as measured by magnetic resonance imaging were observed. It is suggested that humoral factors removed by plasmapheresis might be involved in the pathogenesis of this nonendocrine exophthalmos.