MR imaging of the brain: Findings in asymptomatic patients with thalassemia intermedia and sickle cell-thalassemia disease

OBJECTIVE. The purpose of this study was to evaluate the spectrum of MR fin dings of the brain in asymptomatic patients affected with thalassemia inter media or sickle cell-thalassemia disease to prevent brain damage by identif ying patients at risk for stroke so that transfusional or pharmacologic tre atment could be implemented. SUBJECTS AND METHODS. Forty-one asymptomatic patients who were younger than 50 years and were affected by minor hemoglobinopathies underwent MR imagin g of the brain. Ischemic lesions were classified as small, medium, or large and as single or multifocal. Atrophic changes were graded subjectively as mild, moderate, or severe. A grade of brain damage was assigned to every pa tient. The frequency and severity of brain damage were correlated with the number of sickle-cell crises per year, hemoglobin level, sickling hemoglobi n level, platelet count, sex, and age. RESULTS. Of the patients with thalassemia intermedia, 37.5% showed asymptom atic brain damage, and 52% of those with sickle cell-thalassemia disease sh owed asymptomatic brain damage. In the thalassemia intermedia group, atroph y was always mild and ischemic lesions were generally small (25%) and singl e (25%). Among the patients with sickle cell-thalassemia disease, 24% had s mall, 16% had medium, and 12% had large ischemic lesions. Multifocal lesion s were twice as common in the patients with sickle cell-thalassemia disease (20%) as in those with thalassemia intermedia (12.5%), Only in the patient s with thalassemia intermedia did the frequency of brain damage increase wi th age. Moreover, brain damage inversely correlated with hemoglobin level i n patients with thalassemia intermedia but not in those with sickle cell-th alassemia disease. Brain damage was more severe in patients with sickle cel l-thalassemia disease who had more crises per year. CONCLUSION. This study suggests that patients with thalassemia intermedia a nd those with sickle cell-thalassemia disease may have asymptomatic brain d amage. Our results suggest that MR imaging is useful in identifying patient s at risk for stroke so that they can be treated with transfusional or phar macologic therapy.