Thalassemia Intermedia and Extrmedullary Hematopoiesis
Iraqi Postgraduate Medical Journal,
2013, Volume 12, Issue 4, Pages 622-625
Excessive ineffective erythropoiesis in thalassemia intermedia may cause paravertebral pseudotumors of extramedullary hematopoiesis. Due to the proximity to the spinal canal, these paravertebral masses carry the risk of severe neurological damage. Treatment strategie include hypertransfusion, radiotherapy, and laminectomy.Hydroxyurea, stimulating fetal hemoglobin synthesis, may represent an alternative therapeutic approach. This presentation report a case of female known to have thalassemia intermedia who presented with abnormal gait with weakness of lower limbs. MRI study showed masses originate from thorax vertebra. Hypertransfusion therapy and splenectomy were followed by regular transfusion (baseline hemoglobin 10 g/dl) and chelation with desferrioxamine. With this treatment, clinical symptoms disappeared, paravertebral ematopoietic masses did not progress, Hydroxyurea therapy was initiated to reduce the required transfusion volume but suppressing concomitantly further expansion of extramedullary hematopoiesis, and finally leading to a reduction of transfusional iron load. Treatment was started with 4 mg/kg per day and stepwise increased to 20 mg/kg per day. . Follow up to the patient done by clinical examination and imaging study to assess the vertebral masses.
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