Resolution of bilateral cystoid macular edema and subfoveal serous retinal detachments after treatment with bortezomib in a patient with "smoldering" multiple myeloma.

OBJECTIVE

To describe a case of a patient with multiple myeloma without extraocular end-organ damage but with cystoid macular edema and macular detachments who was treated with bortezomib and dexamethasone. There was a complete resolution of retinal and subretinal fluid and significant improvement of vision.

METHODS

The patient's ocular disease was monitored with visual acuity, dilated fundus examinations, and optical coherence tomography before, during, and after treatment. The patient in this case report was a 43-year-old African American man with a medical history of untreated, "smoldering" multiple myeloma, hypertension, hyperlipidemia who presented to our clinic with progressive painless loss of vision in both eyes over 6 weeks.

RESULTS

Before treatment with bortezomib and dexamethasone, the patient had complaints of confusion, muscle stiffness, joint pain, and 20-lb unintentional weight loss; however, he did not have hypercalcemia, renal insufficiency, anemia, or bone lesions typical of active multiple myeloma. The bilateral cystoid macular edema and subfoveal neurosensory retinal detachments, noted on presentation and confirmed by optical coherence tomography, completely resolved over the course of treatment with bortezomib and dexamethasone.

CONCLUSIONS

This case of bilateral cystoid macular edema and subfoveal neurosensory retinal detachments is remarkable for both its presentation and response to therapy. The macular edema and macular detachments along with nonspecific complaints of confusion, muscle stiffness, joint pain, and weight loss were the presenting signs and symptoms; signs typically used as guides to initiate treatment for multiple myeloma were not present. Macular edema in the context of paraproteinemia is usually associated with Waldenstrom's macroglobulinemia and has classically been reported as "silent" with respect to fluorescein angiography. Our patient has multiple myeloma and demonstrated leakage on fluorescein angiography. The case is also notable in that there was improvement in visual acuity and restoration of normal macular anatomy after receiving eight cycles of bortezomib and dexamethasone. Bortezomib, a chemotherapeutic agent used to treat refractory or rapidly advancing multiple myeloma, had been used previously to treat similar maculopathy in Waldenstrom disease along with plasmapheresis with resolution of macular edema and improvement in visual acuity. Our patient with multiple myeloma did not require plasmapheresis for significant clinical improvement. Treatment with bortezomib and dexamethasone alone was sufficient to clear the bilateral cystoid macular edema and subretinal fluid.