Retinopathy secondary to anemia from myeloid metaplasia in polycythemia vera

Polycythemia vera is a chronic clonal disorder associated with excessive proliferation of erythrocytes, leukocytes, and thrombocytes, as well as an accompanying splenomegaly. Ocular manifestations of polycythemia vera include occipital cortex transient ischemic attacks, transient monocular blindness, vaso-occlusive disease, and retinal hemorrhages.

A 56-year old man with longstanding polycythemia vera sought treatment for a chief symptom of blurred vision in the left eye and a red tinge to things first noticed on awakening that morning. He had preretinal and intraretinal hemorrhages and was subsequently found to be severely anemic as a result of postpolycythemic myeloid metaplasia after years of phlebotomy. Splenectomy controlled his anemia and thrombocytopenia, allowing transient improvement of the retinal hemorrhages. Acute leukemia subsequently developed and the patient died 7 weeks after initial examination.

In this case, preretinal and intraretinal hemorrhages were found in a patient with longstanding polycythemia vera. The exact origin of these hemorrhages is uncertain. They are probably secondary to anemia, but the possibility that they are sites of extramedullary hematopoiesis must be considered. The appearance of retinal hemorrhages warrants careful investigation to rule out diabetes, hypertension, and anemia, as well as the various other blood dyscrasias.