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Polycethemia vera presenting as central retinal vein occlusion

Author: Salma El moataz billah (Morocco)

Co-authors: Sofia Bousseta, Adil Mchachi, Leila Benhmidoune, Abdelouahed Chakib, Ryad Rachid, Mohammed El Belhadji

Purpose

Polycythemia vera is a chronic myeloproliferative disorder characterized by increased red blood cell mass. The resultant hyperviscosity of the blood predisposes such patients to thrombosis.There are scarce literature about ocular involvement in polycethemia vera. We report the case of polycethemia vera presenting as an unilateral retinal central vein occlusion.

Setting/Venue

The patient was admitted to the ophtalmology departement of 20 Aout 1953 university hospital in Casablanca

Methods

A 55 years old patient with no premorbidities, presented to the ophtamology emergency departement for a painless sudden vision loss in the left eye upon awakening. Ophtalmologic examination revealed a central retinal vein occlusion aspect in the affected eye. Fluorescein angiography, macular optical coherence tomography,12-lead electrocardiogram,transthoracic echocardiogram, bilateral carotid artery Doppler, comprehensive laboratory testing, and genetic testing were performed.

Results

Systemic evaluation revealed no abnormalities. Blood parameters were suggestive of polycythemia-hemoglobin 17.50 g%, packed cell volume 56.10%, red blood cell 7.80 millions/mm 3, total white blood cell count 14,200/mm 3, platelets 673,000/mm 3, erythrocyte sedimentation rate 5 mm at the end of 1 h. The patient was referred to the hematology departement. The positive JAK2 V617F mutation test alongside the other biological findings confirmed the diagnosis of polycythema vera. The patient received aspirin, hydroxyurea and underwent aflibercept intravitreal injections for macular oedema.

Conlusions

Ophthalmic effects of polycythemia vera related to hyperviscosity include transient ischemic attacks of the occipital cortex, transient monocular blindness, vaso-occlusive disease, and retinal hemorrhages. Monocular visual loss due to polycythemia vera has been attributed to arteriospasm, retinal artery occlusion due to atherosclerosis or platelet emboli. Uncommon conditions such as PV may rarely present to the ophthalmologist first, with ocular events preceding the systemic manifestations. In the present case, potential life-threatening systemic complications of PV were averted due to the prompt diagnosis and treatment. This case highlights the importance of thorough systemic and haematological workup in patients with CVAO.

Financial Disclosure

No financial interests to disclose

Comments

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