Non-Surgical Management of Progressive Paraparesis due to Diffuse Extramedullary Hematopoiesis (EMH) in beta-Thalassemia Intermedia

Abstract

Background: Thalassemia intermedia is mild form of thalassemia that usually present later in life and transfusion is occasionally needed in these patients. Erythropoiesis is increased in thalassemia causing erythroid marrow hyperplasia as well as EMH which is a common compensatory mechanism for chronic anemia found in such patients. EMH usually occures in liver, spleen, kidneys and as paravertebral region. It is observed in 30%–60% of patients with thalassemia intermedia while involvement of the paraspinal region has a prevelance of about 11%–15% among these cases.

Case presentation: A 26 year old man with beta thalassemia intermedia developed pain in low back region with progressive paresthesia because of multifocal EMH which occurred all around the spinal canal with compression over the cord. This case was a recurrent one who had a previous episode of disease 6 years ago. The patient could be managed with repeated episodes of radiation to the spine and all the lesions disappeared in the last follow up.

Conclusion: Reporting this unique case and its characteristics, different aspects of a better treatment protocol in similar cases will be discussed. Management of recurrent neurological deficit due to EMH compressing the spinal cord has not been discussed in the literature previously.