Spinal cord compression and extramedullary hematopoiesis in young Egyptian beta-thalassemia patients.
The problem of spinal cord compression (SCC) related to extramedullary hematopoiesis (EMH) in beta-thalassemia (beta-thal) patients, both clinically and radiologically and its correlation with laboratory parameters of anemia and hemosiderosis was assessed. Sixty beta-thal patients were included and divided into group I: 40 beta-thal major patients (beta-TM), aged 7-30 years with a mean age of 15 +/- 5.3 years, group II: 20 beta-thal intermedia patients (beta-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 beta-TI compared to beta-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 beta-TI patients, and was related to inadequate chelation, high serum ferritin and inadequate transfusion therapy.
Tantawy
Azza A G
AA
Department of Pediatrics, Ain Shams University, Cairo, Egypt. azazaghoul@yahoo.com
Adly
Amira A M
AA
Mahdy
Sameh A R
SA
Kamel
Ghada Z
GZ
D000293:Adolescent; D000328:Adult; D000740:Anemia / Q000209:etiology; D001803:Blood Transfusion; D002648:Child; D005293:Ferritins / Q000097:blood; D006411:Hematopoiesis, Extramedullary; D006454:Hemoglobins / Q000032:analysis; D006486:Hemosiderosis / Q000209:etiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D013117:Spinal Cord Compression / Q000175:diagnosis / Q000209:etiology*; D014057:Tomography, X-Ray Computed; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications*
D006454:Hemoglobins; D005293:Ferritins