Bilateral, persistent serous macular detachments with Waldenström's macroglobulinemia

Waldenström's macroglobulinemia is a rare, malignant lymphoplasmacytic disorder characterized by the monoclonal proliferation of immunoglobulin type M (IgM) producing B-lymphocytes. Ocular manifestations of Waldenström's macroglobulinemia have been described in association with the conjunctiva, cornea, uvea, retina, and periocular adnexa. Only rarely have macular findings, particularly serous macular detachments, been described in the presence of monoclonal hypergammaglobulinemias. The majority of reports to date have documented resolution of these serous macular detachments after treatment with blood plasmapheresis. This report presents a case of bilateral, persistent serous macular detachments in the presence of Waldenström's macroglobulinemia.

A 53-year-old black man presented with gradual bilateral reduction in visual acuity over the last 2 months. He revealed having been diagnosed with Waldenström's macroglobulinemia approximately 1 month before presentation. He also reported having undergone multiple plasmapheresis treatments in conjunction with systemic chemotherapy over the same period. Funduscopic and fluorescein angiographic examination revealed bilateral, serous macular detachments in the presence of mild venous stasis retinopathy secondary to serum hyperviscosity. Subsequent funduscopic evaluations and serial optical coherence tomography readings confirmed the persistence of the serous detachments despite multiple plasmapheresis treatments.

Although the funduscopic manifestations of excess serum immunoglobulins are typically the sequelae of circulatory stasis, the serum hyperviscosity induced in Waldenström's macroglobulinemia has been shown on rare occasions to induce serous macular detachments. Physical elimination of the excess macroglobulins through plasmapheresis is quite effective in resolving the neurosensory detachments induced by Waldenström's macroglobulinemia. Only a fraction of neurosensory detachments resulting from Waldenström's macroglobulinemia persist despite multiple plasmapheresis treatments. Novel angiographic evidence of retinal pigment epithelium atrophy beneath the area corresponding to the serous detachment provides a plausible explanation for unresponsive nature of this presentation. Identification of this rare variant of the classic angiographically silent macula known to occur with Waldenström's macroglobulinemia can aid the clinician in determining a patient's potential response to plasmapheresis and their final visual prognosis.