Spinal Cord Compression And Extramedullary Hematopoiesis in Young Egyptian β-thalassemia Patients

The problem of spinal cord compression (SCC) related to extramedullary hematopoiesis (EMH) in β-thalassemia (β-thal) patients, both clinically and radiologically and its correlation with laboratory parameters of anemia and hemosiderosis was assessed. Sixty β-thal patients were included and divided into group I: 40 β-thal major patients (β-TM), aged 7-30 years with a mean age of 15 ± 5.3 years, group II: 20 β-thal intermedia patients (β-TI) aged 6-20 years with a mean age of 13 ± 4.6 years. They were subjected to neurological examination, thoracic and lumbosacral computed tomography (CT) and magnetic resonance imaging (MRI). Spinal EMH was found in 13.3% of the thalassemic patients with a higher incidence in β-TI compared to β-TM patients (p = 0.03). Evidence of spinal EMH was associated with higher serum ferritin (p < 0.0001), lower pre transfusion hemoglobin (Hb) (p = 0.002) and lower transfusion index (p = 0.01). Extramedullary hematopoiesis was more evident in young β-TI patients, and was related to inadequate chelation, high serum ferritin and inadequate transfusion therapy.