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Paraplegia with thalassaemia.

C V David, P Balasubramaniam

    The Australian and New Zealand Journal of Surgery
    |June 1, 1983
    PubMed
    Summary
    This summary is machine-generated.

    Extramedullary hematopoiesis, the formation of blood cells outside bone marrow, can cause spinal cord compression in patients with beta-thalassemia intermedia. Surgical removal of this tissue relieved symptoms in a reported case.

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    Area of Science:

    • Hematology
    • Neurology
    • Spinal Surgery

    Background:

    • Thalassemias are inherited blood disorders characterized by reduced hemoglobin production.
    • Beta-thalassemia intermedia is a moderate form of the disease.
    • Extramedullary hematopoiesis (EMH) is a known complication in thalassemia, typically occurring in the spleen and liver.

    Observation:

    • A 28-year-old woman with beta-thalassemia intermedia developed progressive paraparesis and incontinence over two weeks.
    • Spinal MRI revealed extramedullary hematopoietic tissue compressing the spinal cord in the epidural space.
    • This presentation of EMH causing spinal cord compression is exceedingly rare.

    Findings:

    • Surgical decompression via laminectomy and removal of the extramedullary hematopoietic tissue resulted in neurological recovery.

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  • Histopathological examination confirmed the removed tissue to be active hematopoietic marrow.
  • This case adds to the limited literature documenting spinal EMH in thalassemia.

    • Implications:

    • Highlights a rare but potentially devastating neurological complication of beta-thalassemia intermedia.
    • Emphasizes the importance of considering EMH in the differential diagnosis of spinal cord compression in thalassemia patients.
    • Surgical intervention can be effective in reversing neurological deficits caused by spinal EMH.