Combined central retinal and posterior ciliary artery occlusion in a young female with primary myelofibrosis and Sweet’s syndrome: A case report

Abstract: Primary myelofibrosis (PMF) is a rare myeloproliferative neoplasm that typically affects older adults but may present aggressively in younger patients. Thromboembolic complications are well recognized, though arterial occlusions are uncommon in this age group. Ocular ischemia associated with systemic inflammatory conditions such as Sweet’s syndrome is exceedingly rare. A 22-year-old female with PMF, diagnosed 18 months earlier and managed initially with ruxolitinib, presented with sudden, complete loss of vision in her left eye. She also had a history of Sweet’s syndrome managed with oral corticosteroids and had received multiple blood transfusions. Ophthalmic evaluation revealed no light perception in the left eye, a pale retina with attenuated vessels, and a cherry-red spot. Optical coherence tomography (OCT) showed hyperreflectivity of the inner retinal layers, and OCT angiography demonstrated markedly reduced perfusion in all vascular plexuses. Carotid Doppler, echocardiography, and ECG excluded embolic sources. Despite ocular massage, anterior chamber paracentesis, acetazolamide, and anticoagulation with apixaban, vision did not recover. One week later, she developed anterior uveitis, keratic precipitates, and elevated intraocular pressure. Fundus showed Amalric’s sign of choroidal infarction and Elschnig spots, confirmed on fluorescein angiography. Inflammation responded to topical and systemic corticosteroids, with normalization of intraocular pressure. This is the first documented case of combined central retinal artery occlusion and posterior ciliary artery occlusion with anterior uveitis in a young PMF patient with Sweet’s syndrome. The case underscores the multifactorial contribution of hypercoagulability, systemic inflammation, and transfusion-related factors in ocular ischemia.