Retinal vein occlusion during flare of multicentric Castleman’s disease
A 63-year-old active male cyclist with HIV (CD4 377/viral load <48 copies/mL) presented with blurred vision and light sensitivity in his left eye for several days while experiencing a flare of MCD disease with fever, tender lymphadenopathy, and profound fatigue. The patient had a history of right eye central retinal vein occlusion with vitreous hemorrhage resulting in significant permanent vision loss 4 years previously. Near that time he initially developed recurrent fevers, he also had cytopenias, hepatosplenomegaly, and tender lymphadenopathy that eventually led to diagnosis of MCD confirmed by lymph node biopsy. The patient declined recommended therapy with rituximab due to share of cost and prolonged remissions between flares. Initiation of valganciclovir 1-year ago resulted in attenuation and reduced frequency of MCD flares. On examination, his visual acuity was 20/200 in the right eye and 20/100 in the left eye. Intraocular pressure in both eyes and blood pressure were normal. Retinal examination in the right eye showed macular atrophy and laser burns after panretinal photocoagulation. The fundus exam of the left eye revealed optic nerve swelling, tortuous veins, cotton-wool spots, and intraretinal hemorrhages, a few of which were white-centered Roth spots (white arrows on ). Fluorescein angiography and spectral-domain optical coherence tomography (SD-OCT) showed delayed perfusion times and macular edema in the left eye, respectively. SD-OCT showed subretinal fluid extending from the optic disc to the fovea (). Hypercoagulability workup demonstrated only mildly increased anti-cardiolipin antibody; there was no evidence of a lupus anticoagulant, prothrombin, or factor V Leiden mutation and the patient had normal antithrombin, protein C, and S. His left eye was treated with intravitreal injection of bevacizumab 1.25 mg in 0.05 mL. After a single injection there was a rapid improvement in visual acuity in his eye. At the last visit 6 months after treatment his visual acuity remained at 20/20 with normal retinal findings (). He also initially received the recommended systemic therapy with rituximab followed by high dose zidovudine with valganciclovir; this resulted in the longest remission to date of systemic MCD flare (>12 months to date).