Recurrent thoracic cord compression due to extramedullary haematopoiesis in thalassaemic patients–A case presentation and literature review

Abstract

Extramedullary haematopoesis (EMH) in thalassaemic patients to result in spinal cord compression is a rare phenomenon. EMH occurs as compensatory sequelae in those with chronic anaemia such as thalassaemia, other congenital haemolytic anaemia, haemoglobinopathies, and myelofibrosis. Common sites of EMH include liver, spleen, kidneys and lymph nodes, but rarely the paravertebral and spinal cord regions. In our literature review, it was found that only case reports and case series have been published. We present a case of recurrent thoracic spinal cord compression caused by EMH in a 32-year-old thalassaemic Chinese man treated with surgical decompression and spinal stabilisation. The key to successful treatment is prompt diagnosis with early clinical suspicion, magnetic resonance imaging and individualised treatment for each patient. Surgical decompression provides immediate neural decompression while subsequent anaemia management may reduce the risk of recurrence. In the present case, a combination therapy of blood transfusion, surgical decompression and radiotherapy has been shown to deliver successful outcomes in such cases.