Background: To report the rare association of peripheral retinal ischemia in a patient with Waldenström's macroglobulinemia.
Case presentation: A 39-year old man with a recent diagnosis of asymptomatic Waldenström's macroglobulinemia (WM) was referred from his physician for ocular evaluation. The fundus examination in his right eye (RE) revealed very mild central vein dilation, while retinal hemorrhages associated with microaneurismal alterations of the vascular plexus were detected at the temporal periphery. Fluoroscein angiography of his RE revealed an extended area of capillary dropout distal to the microaneurismal lesions. In our patient with WM an extensive area of capillary non-perfusion, in the absence of severe involvement of the posterior pole was documented; this association to the best of our knowledge has never been reported before.
Conclusion: Although the incidence of the disease is rare, meticulous examination of the retinal periphery should be performed in all patients with WM and vice versa the differential diagnosis of peripheral retinal ischemia of unknown origin should include an investigation to rule out asymptomatic Waldenström's macroglobulinemia.