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Published on: September 6, 2017
Intracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma
Noah M Nichols1, Ashia M Hackett1, Alejandro Carrasquilla1
1Department of Neurological Surgery, Icahn School of Medicine at Mount Sinai, New York City, New York, United States.
Background:
Beta-thalassemia is an inherited hemoglobinopathy, whereby reduced or absent expression of beta-globin genes causes impaired erythropoiesis. Extramedullary hematopoiesis (EMH) occurs in 1% of all patients with beta-thalassemia major receiving regular transfusions and is exceedingly rare intracranially.
Case Description:
We report a case of a male in his 20s with beta thalassemia who presented with head trauma found to have intracranial EMH mimicking multiple extra-axial hematomas. Making the correct diagnosis was critical in avoiding prolonged neuromonitoring and unnecessary interventions.
Conclusion:
Intracranial extramedullary hematopoietic pseudotumor is an exceedingly rare entity and seldom appears in a neurosurgeon's differential diagnosis. This case illustrates how this condition can easily mimic an acute intracranial hemorrhage in a patient with beta-thalassemia who presents with head trauma. We review the topic to further inform clinicians who may encounter this condition in their practice.
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