Painful spinal cord compression as a complication of extramedullary hematopoiesis associated with beta-thalassemia intermedia

Abstract

Background: Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare manifestation of thalassemia. General therapeutic recommendations do not exist.

Case report: We report a 23 year old male with beta-thalassemia intermedia and back pain resistant to analgetic medication. Multiple extradural mass lesions on MRI of the lumbar spine were identified. Hypertransfusion was initiated with no relief of symptoms. Low dose irradiation with 20 Gy had to be added to achieve adaequate response.

Result: With the combination of transfusion therapy and irradiation a rapid and long-lasting relief of symptoms was achieved. 12 month post irradiation the patient is free of pain and neurological symptoms.

Conclusion: Clinical awareness of this phenomenon and knowledge of the available treatment option is essential for successful outcome. In treatment refractory symptoms irradiation could successfully contribute to a rapid therapeutic response.