Compressive Optic Neuropathy in Thalassemia: A Rare Ophthalmic Consequence of Extramedullary Hematopoiesis

Abstract

Extramedullary hematopoiesis (EMH) is a compensatory physiological proliferation of hematopoietic elements outside the bone marrow as a result of insufficient bone marrow erythropoiesis. It is found in various hematologic disorders (e.g., as sickle cell anemia, thalassemia, myelofibrosis) especially those with chronic anemias. It can affect in any organs, thus producing numerous complications. However, compressive optic neuropathy has been rarely reported. The authors present a case report of an 18-year-old man who was known to be beta thalassemia/Hb E disease with the presentation of compressive optic neuropathy with an excellent response to combination treatment of blood transfusions and low-dose radiotherapy. These findings reflect that beta thalassemia/Hb E or thalassemia intermedia patients should have periodic comprehensive ophthalmic assessment especially those with inadequate blood transfusions. Moreover, favorable visual outcome can be achieved with early recognition and prompt management with blood transfusions combined with low-dose radiotherapy.