Management of spinal cord compression caused by extramedullary hematopoiesis in beta-thalassemia

Abstract

Thalassemia is a chronic hemolytic anemia, endemic around the Mediterranean basin. Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that can involve many organs or tissues, including the epidural space, leading to a spinal cord compression syndrome. In almost all cases, the clinical and MR patterns are those of a lower dorsal spinal cord progressive compression secondary to an epidural expanding process. The management still remains controversial, including mainly blood transfusions, decompressive surgery and radiotherapy. We present a case of EMH in a thalassemic adolescent boy presenting with a T12 level of compression, treated successfully with blood transfusion, surgical decompression and radiotherapy.