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  • Title: [Spinal cord compression caused by extramedullary hematopoiesis foci in the course of thalassemia].
    Author: Rey J, Gagliano R, Christides C, Pillard E, Magnan F, Tourniaire P, Arwidson I, Raymond-Gelle MC, Boulat O, Arlaud J.
    Journal: Presse Med; 2001 Sep 29; 30(27):1351-3. PubMed ID: 11675925.
    Abstract:
    BACKGROUND: Extramedullary haematopoiesis is a physiological response to chronic anemia, observed frequently during homozygous thalassemia. It is usually asymptomatic but can be manifested by compression of adjacent organs, particularly the spinal cord. CASE REPORT: A 44-year-old woman diagnosed with thalassaemia intermedia, was admitted for difficulties to walk and sphincter disturbances. Neurologic examination suggested spinal cord compression, which is confirmed by dorso-lumbar resonance magnetic imaging. The histology obtained by laminectomy led to the diagnosis of extramedullary hematopoiesis related to thalassemia. A radiotherapy enabled with good outcome. DISCUSSION: Spinal cord compressions by extramedullary hematopoiesis during thalassemia are uncommon (75 cases in the literature) but can induce severe sequelae if the diagnostic is not rapidly obtained. Magnetic resonance imaging is the gold standard allowing precise diagnosis and spreading of extramedullary hematopoiesis. Radiotherapy and more recently hydroxyurea are the first line treatment. CONCLUSION: This observation recalls that extramedullary hematopoiesis is a differential diagnostic of spinal cord compression in patients with thalassemia. A screening of paravertebral localization of extramedullary hematopoiesis should be performed in high risk thalassemic patients.
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