2,3 If severe, visual acuity can be affected.

This consists of retinal venous dilation and beading, increased venous tortuosity, peripheral and central retinal hemorrhages, retinal and optic disc edema, and retinal vascular occlusions. As a result, hyperviscosity-related retinopathy may develop. In Waldenstrom’s macroglobulinemia, the increase in serum viscosity leads to microvascular sludging with decreased blood flow to the retina and increased intravascular pressure within the retinal venous circulation. 1 Serum hyperviscosity can also lead to the hyperviscosity syndrome, which may cause fatigue, weight loss, neurological symptoms, epistaxis, gastrointestinal hemorrhages, congestive heart failure, and retinopathy. Manifestations of the disease are secondary to infiltration of the bone marrow and extramedullary sites and elevated serum levels of IgM, and include pancytopenia, organomegaly, neuropathy, and effects of serum hyperviscosity. Waldenstrom’s macroglobulinemia is a B-cell lymphoproliferative disorder with lymphoplasmacytic infiltration of the bone marrow and lymphatic tissue leading to overproduction of the monoclonal immunoglobulin protein IgM. Serum viscosity at this time was normal at 1.7 cP and his IgM was 1100 mg/dl. The posterior segment of the left eye remained unremarkable (Figure 5B). On the patient’s most recent follow-up examination, approximately 7 years after his initial visit and one year after his completion of chemotherapy, visual acuity measured 20/20 in both eyes, and the venous dilation and peripheral retinal hemorrhages in the right eye had resolved (Figure 5A). This subsequently led to a significant decrease in the patient’s IgM level and normalization of serum viscosity. Two years later, he underwent additional treatment with 6 cycles of chemotherapy. He had 5 treatments with rituximab along with plasmapheresis at variable intervals. Serum viscosity at this time was 2.6cP and his IgM level was 4700mg/dl.Īpproximately 4 years after his initial visit, the patient began treatment of his Waldenstrom’s macroglobulinemia due to anemia, fatigue, and body aches.

FA in the left eye revealed a lesser degree of peripheral nonperfusion and microaneurysms temporally (Figure 4B). Fluorescein angiography at this follow-up visit displayed less hypofluorescence from blockage but increased peripheral nonperfusion and microaneurysms temporally (Figure 4A). The posterior segment of the left eye was unremarkable (Figure 3C). A small collateral vessel was also visible at the optic nerve (Figure 3B). At 32 months of follow-up, the right eye posterior segment still had venous dilation but the peripheral retinal hemorrhages had decreased in number. On all of the patients’ subsequent follow-up examinations, the visual acuity remained stable at 20/20 bilaterally. At presentation, his serum viscosity was 2.4 cP and his IgM level was 4200 mg/dl. Past medical history withheld for the purpose of discussion was that our patient had a known diagnosis of Waldenstrom’s macroglobulinemia with increased production of IgM. FA of the left eye was unremarkable (Figure 2D).Īdditional Case History and Clinical Course: Fluorescein angiography (FA) of the right eye was significant for spots of hypofluorescence in the macula and temporal and inferior retinal periphery corresponding to blockage from the scattered retinal hemorrhages and peripheral nonperfusion along with several small microaneurysms temporally (Figures 2A – 2C).

The posterior segment of the left eye showed mild venous dilation (Figures 1C and 1D). There was also very mild dilation but no increased tortuosity of the retinal veins and several larger retinal hemorrhages in the temporal and inferior mid-periphery (Figure 1B). On posterior segment examination of the right eye, there was optic nerve hyperemia and a few small dot-blot hemorrhages in the macula (Figure 1A). Intraocular pressure and anterior segment examination were normal in both eyes. On examination, best-corrected visual acuity was 20/20 bilaterally. His only medications were alendronate, glucosamine, calcium, vitamin D, and low-dose aspirin. His past ocular, family, and social history were all noncontributory. The patient denied any decreased vision, pain, or other ocular symptoms. A 57 year-old Asian man was referred for evaluation of retinal hemorrhages in the mid-periphery of the right eye.

Dot blot hemorrhage eye