Subacute compressive myelopathy secondary to extramedullary hematopoiesis

Abstract

A 25-year-old man presented with subacute onset paraparesis with sphincter disturbance. His past medical history unvealed b-thalassemia intermedia (compound heterozygote IVS 1-5) and hereditary persistence of fetal hemoglobin type 2. His neurological examination disclosed a complete spinal cord syndrome without clear thoracic sensory level. Spinal cord imaging showed severe thoracic spinal stenosis secondary to paraspinal extramedullary hematopoiesis ( Figure). Spinal cord compression is a leading cause of myelopathy in patients with multiple progressive paravertebral masses in chronic extramedullary hematopoiesis 1 , described in hereditary hemoglobinopathies, lymphoproliferative disorders, myelofibrosis and polycythemia vera