Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria

Abstract

Aims: To describe a Central Retinal Vein Occlusion Secondary to Paroxysmal Nocturnal Hemoglobinuria. Presentation of Case: A 25 years old, male, student, in regular follow-up in the Hematology sector due to Paroxysmal Nocturnal Hemoglobinuria in regular use of Eculizumab. He reports a month ago that he suddenly noticed, upon waking up in the morning, blurring and decreased visual acuity in her left eye. Discussion: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder characterized by hemolysis, thrombosis, and bone marrow failure caused by defective expression of glycosylphosphatidylinositol-anchored (GPI-anchored) complement inhibitors. Most commonly, PNH is caused by the loss of PIGA function, which is necessary for GPI biosynthesis. Conclusions: Patients with hemolytic anemia, unexplained thrombosis, especially in uncommon sites, cytopenias and bone marrow failure syndromes, dysphagia, and unexplained abdominal pain should be screened for PNH. PNH patients can benefit greatly from the treatments available, with a reduction in the risk of serious sequelae and a considerable improvement in their quality of life. So that the patient continues to be followed up in the ophthalmology department, with decreased visual acuity ipsilateral to the lesion, with a relative afferent pupillary defect on that side.