Extramedullary Hematopoiesis Presenting with Thoracic Spinal Cord Compression in a Young Adult with Thalassemia Major: A Case Report.

Abstract

Background: Extramedullary hematopoiesis (EMH) refers to the production of blood cellular components at sites other than the bone marrow, namely liver, spleen, and lymph nodes. The common sites associated with this condition are the liver, spleen, and lymph nodes whereas the common conditions associated with it are myelofibrosis, myelodysplasia, thalassemia, sickle cell anemia, and polycythemia vera.

Case description: This report describes a young male with thalassemia major, who presented with symptomatic cord compression due to a thoracic intraspinal lesion. It was surgically excised and diagnosed as a case of EMH. The boy recovered fully and has been asymptomatic for six months now.

Conclusion: The occurrence of EMH in the thoracic spine is uncommon, whereas symptomatic cord compression as a result of it is even more unusual. Magnetic resonance imaging (MRI) is the diagnostic imaging of choice and treatment options that can be offered are surgical decompression, radiotherapy, hydroxyurea, and transfusion of packed red blood cells (RBCs).