Idiopathic myelofibrosis causing inflammatory central retinal vein occlusion mimicking frosted branch angiitis

Abstract

Primary idiopathic myelofibrosis is a clonal disorder arising from the neoplastic transformation of early haematopoietic stem cells leading to abnormal fibrous tissue within bone marrow. We present a 51-year-old man complaining of sudden loss of vision in the right eye along with multiple superficial retinal haemorrhages and perivascular infiltration. Fundus fluorescein angiography and optical coherence tomography confirmed the diagnosis of inflammatory central retinal vein occlusion with macular oedema which mimicked frosted branch angiitis. Laboratory tests and bone marrow biopsy confirmed underlying systemic disease to be Janus kinase 2 mutation positive primary idiopathic myelofibrosis. He received one intravitreal injection of antivascular endothelial growth factor along with dexamethasone for his ocular condition. In view of his systemic disease, he was started on systemic chemotherapy and oral corticosteroid which helped in stabilising the eye condition.