Compressive Myelopathy Due to Extramedullary Hematopoiesis in Nontransfusion Dependent E Beta-thalassemia

Abstract Extramedullary hematopoiesis (EMH) occurs in organs other than bone marrow. Apart from liver and spleen, EMH occurs rarely and is often asymptomatic, but can sometimes present with tumor-like masses and lead to symptoms. Paraspinal involvement warrants special attention due to the paralyzing sequel. This report describes a case of a thalassemia patient with paraspinal elongated long tumor, with the description of the clinical manifestations, imaging, pathology, diagnosis, and treatment of EMH. A 23-year-old male, known case of E beta-thalassemia since childhood (1.5 years of age), transfusion-dependent thalassemia (TDT) converted to non-TDT in 2013 when the patient was put on hydroxyurea, followed by thalidomide, and was maintaining hemoglobin (Hb) around 10 g/dl without iron overload. He presented with fast progressive paraplegia with features of compressive myelopathy. Magnetic resonance imaging spine was suggestive of lobulated, large intraspinal extradural soft tissue lesion involving posterior elements of the dorso-lumbar region, as well as along the bilateral chest wall, insinuating into the thoracic pleura and extending into the spinal canal and causing compression over the spinal cord from D4 to D8 vertebral levels. Dorsal thoracic decompression with D4-D8 laminectomy was performed, and this was followed by hydroxyurea, thalidomide, and red blood cell transfusion to maintain Hb >10 g/dl. Microscopy showed the hematopoietic elements. In conclusion, EMH is a rare manifestation and should be distinguished from other neoplasms. With masses, characteristic clinical features, and radiologic appearance, EMH must be considered in patients with thalassemia.