Bêta-thalassémie intermédiaire compliquée d’une compression médullaire. Étude de trois observations et méta-analyse

Abstract

Extramedullary hematopoiesis is common in patients who suffer from beta-thalassemia. Extramedullary hematopoiesis is a compensatory mechanism of chronic anemia. Although the diagnosis is relatively easy, management is still the subject of considerable controversy. The aim of the present paper is to report three cases of spinal cord compression due to beta-thalassemia followed by a meta-analysis. In one of the three cases reported, spinal compression revealed beta-thalassemia. Forty-three cases have been reported in the last 10 years, i.e., four cases per year. The diagnosis was made by CT and myelography and confirmed by histology after surgical treatment. Two patients were followed in the hematology department. Young patients accounted for 95.45% of cases. The time to diagnosis was 3.33 months (3 days to 12 months). The most sensitive exam for diagnosis is magnetic resonance imaging before surgery. The MR image shows a hypointense lesion on T1-weighted sequences in 30.43% of cases or isointense on T2-weighted sequences in 34.78% of cases, and hypointense on T1-weighted sequences enhanced with gadolinium in 42.85% of cases. When MRI is absent, as in our patients, myelography can provide the diagnosis. The vertebrae are enlarged and translucent in 85.71% of cases with a hypodense lesion compressing the spinal cord. Laboratory tests show chronic microcytosis hemolytic anemia in 97.17% of cases. The diagnosis was established by histology performed after surgical removal. The patients recovered 97.5% of their neurologic deficiency after specific treatment. The mean follow-up was 2 years (4 weeks to 15 years).