We present a case of a patient with hyperviscosity-related retinopathy secondary to Waldenstrom macroglobulinemia that had dramatic improvement in both ocular and systemic symptoms following plasmapheresis. The resolution of his white-centered peripheral lesions was sustained nearly one and a half years later (Figures 1(e) and 1(f)). The patient was started on five months of rituximab and bendamustine. Visual acuity improved from 20/25 OU to 20/20 OU. Pre- and posttreatment en face OCT (Figures 3(a) and 3(d)) and spectral domain OCT (Figures 4(a) and 4(b)) over the white-centered peripheral lesions showed significant resolution of the parafoveal outer nuclear layer hyperreflective material.Ĭolor fundus photos and spectral domain OCT over white-centered peripheral lesions over the right nasal fovea three days before (a) and one week after (b) plasmapheresis show significant resolution of the outer nuclear layer hyperreflective material.įollowing plasmapheresis, the patient noticed improvements in metamorphopsia, headaches, and dizzy spells. After treatment, capillary density decreased from 47.62% to 45.35%, and large vessel density decreased from 18.87% to 10.16%. A color-coded density map was created for a rapid qualitative assessment (Figures 3(c) and 3(f)). Next, computation of capillary and large vessel densities was performed. Segmentation of capillaries was performed after removal of the large blood vessels using local thresholding (MATLAB 2018b MathWorks, Natick, MA). After treatment, capillary density decreased from 47.62% to 45.35% and large vessel density decreased from 18.87% to 10.16%. Capillaries are color-coded large vessels are excluded and shown in white. (c, f) Capillary density maps before (c) and after (f) plasmapheresis. Qualitatively, there is a noticeable decrease in vessel diameter.
(b, e) Full vascular OCT-A layer before (b) and after (e) plasmapheresis. One week after treatment, there was a significant decrease in the hyperreflective lesion nasal to the fovea.
(a, d) OCT reflectance full layer before (a) and after (d) plasmapheresis. OCT angiography of the right retina, nasal to the fovea, before and one week after finishing plasmapheresis. His oncologist started one round of intravenous immunoglobulin, followed by three rounds of plasmapheresis. The patient also started to notice “waviness,” episodic dizziness, and headaches. Fluorescein angiography from this time is shown in Figure 2. Upon reexamination, new vitreous hemorrhage, white-centered peripheral lesions, and increasing vascular tortuosity were noticed (Figures 1(a) and 1(b)).
Several months later, the patient was diagnosed by his oncologist with Waldenstrom macroglobulinemia. Case ReportĪ 55-year-old man with an ocular history of posterior vitreous detachment, lattice degeneration with atrophic hole, and glaucoma suspect was referred for evaluation of retinal vascular tortuosity. OCT-A can aid in the noninvasive evaluation of retinal perfusion deficits and potentially guide treatment courses by monitoring microvascular integrity. Though treating hyperviscosity improves the clinical appearance of the retina, some patients ultimately suffer from decreased vision due to ischemic maculopathy. Previous studies have shown that as serum viscosity and serum IgM levels rise, both peripheral hemorrhages and venous dilation increases. The resultant retinal hypoxia of the vascular endothelial cells leads to vascular tortuosity, retinal hemorrhage, exudates, and retinal vein occlusion.
Hyperviscosity is thought to cause autoregulatory venous dilation, subsequent venous stasis, and a rise in intravascular pressure.
It results in unregulated production of IgM which increases the viscosity of the blood. Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma, with an incidence of 2-5 cases per million person years, median age of diagnosis of approximately 69 years old, and a male-to-female ratio of approximately 2 : 1. This technique can be used to guide treatment and surveillance for patients with hyperviscosity-related retinopathy.
Applying image analysis software to before and after OCT-A images, the authors were able to show an objective decrease in retinal capillary and large vessel density following plasmapheresis. The authors present a case of a patient with Waldenstrom macroglobulinemia who had resolution of white-centered peripheral retinal lesions and parafoveal outer nuclear layer hyperreflective material following plasmapheresis. While plasmapheresis is well known to significantly improve both retinal findings and systemic manifestations associated with Waldenstrom macroglobulinemia, few reports exist documenting changes in optical coherence tomography angiography (OCT-A).