A 25-year-old man presented with subacute onset paraparesis with sphincter disturbance. His past medical history unvealed β-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 hematopoiesis11 . Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia. Eur Spine J 2010;19:871-878., described in hereditary hemoglobinopathies, lymphoproliferative disorders, myelofibrosis and polycythemia vera22 . Haidar R, Mhaidli H, Musallam KM, Taher AT. The spine in beta-thalassemia syndromes. Spine 2012;37:334-339..
Non-contrast-enhanced sagittal MRI of the spine at thoracic level disclosing multiple solid masses in the paravertebral spine with intermediate signal on T2-weighted (A; asterisks) and on T1-weighted images (B; arrows). Contrast-enhanced T1-weighted sagittal MRI of the spine showing enhancement of the solid masses described in A and B (C; filled arrowhead). Axial T2-weighted MRI of the spine evinced severe thoracic spinal stenosis and multiple paravertebral masses (D; non-filled arrowhead).
References
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1Haidar R, Mhaidli H, Taher AT. Paraspinal extramedullary hematopoiesis in patients with thalassemia intermedia. Eur Spine J 2010;19:871-878.
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2Haidar R, Mhaidli H, Musallam KM, Taher AT. The spine in beta-thalassemia syndromes. Spine 2012;37:334-339.
Publication Dates
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Publication in this collection
Sept 2014
History
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Received
12 Apr 2014 -
Reviewed
01 May 2014 -
Accepted
16 June 2014
