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Incomplete paraplegia caused by extramedullary hematopoiesis in a patient with thalassemia intermedia
Nurhasyimah Hisamud-Din1,2, Nadia Mohd Mustafah2,3, Aishah Ahmad Fauzi2
1Department of Rehabilitation Medicine, Hospital Tuanku Ja'afar, Seremban, Negeri Sembilan, Malaysia.
Introduction:
Extramedullary hematopoiesis (EMH) is the production of blood cell precursors outside the bone marrow that occur in various hematological diseases. In patients with thalassemia intermedia, ineffective erythropoiesis drives compensatory EMH in the liver, pancreas, pleura, spleen, ribs and spine.
Case Presentation:
We describe a patient with thalassemia intermedia who presented with acute neurological symptoms caused by paraspinal EMH, which responded well to combination therapy of steroid, hypertransfusion, laminectomy and excision of pseudotumor and hydroxyurea therapy to boost the formation of fetal haemoglobin.
Discussion:
Prompt recognition of EMH based on clinical presentation and typical radiological findings should be made. Early treatment is recommended to prevent irreversible damage to the spinal cord.
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