OBJECTIVE: The purpose of this study was to evaluate the spectrum of MR findings of the brain in asymptomatic patients affected with thalassemia intermedia or sickle cell-thalassemia disease to prevent brain damage by identifying patients at risk for stroke so that transfusional or pharmacologic treatment could be implemented. SUBJECTS AND METHODS: Forty-one asymptomatic patients who were younger than 50 years and were affected by minor hemoglobinopathies underwent MR imaging 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 patient. The frequency and severity of brain damage were correlated with the number of sickle-cell crises per year, hemoglobin level, sickling hemoglobin level, platelet count, sex, and age. RESULTS: Of the patients with thalassemia intermedia, 37.5% showed asymptomatic brain damage, and 52% of those with sickle cell-thalassemia disease showed asymptomatic brain damage. In the thalassemia intermedia group, atrophy was always mild and ischemic lesions were generally small (25%) and single (25%). Among the patients with sickle cell-thalassemia disease, 24% had small, 16% had medium, and 12% had large ischemic lesions. Multifocal lesions were twice as common in the patients with sickle cell-thalassemia disease (20%) as in those with thalassemia intermedia (12.5%). Only in the patients with thalassemia intermedia did the frequency of brain damage increase with age. Moreover, brain damage inversely correlated with hemoglobin level in patients with thalassemia intermedia but not in those with sickle cell-thalassemia disease. Brain damage was more severe in patients with sickle cell-thalassemia disease who had more crises per year. CONCLUSION: This study suggests that patients with thalassemia intermedia and those with sickle cell-thalassemia disease may have asymptomatic brain damage. Our results suggest that MR imaging is useful in identifying patients at risk for stroke so that they can be treated with transfusional or pharmacologic therapy.
OBJECTIVE: The purpose of this study was to evaluate the spectrum of MR findings of the brain in asymptomatic patients affected with thalassemia intermedia or sickle cell-thalassemia disease to prevent brain damage by identifying patients at risk for stroke so that transfusional or pharmacologic treatment could be implemented. SUBJECTS AND METHODS: Forty-one asymptomatic patients who were younger than 50 years and were affected by minor hemoglobinopathies underwent MR imaging 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 patient. The frequency and severity of brain damage were correlated with the number of sickle-cell crises per year, hemoglobin level, sickling hemoglobin level, platelet count, sex, and age. RESULTS: Of the patients with thalassemia intermedia, 37.5% showed asymptomatic brain damage, and 52% of those with sickle cell-thalassemia disease showed asymptomatic brain damage. In the thalassemia intermedia group, atrophy was always mild and ischemic lesions were generally small (25%) and single (25%). Among the patients with sickle cell-thalassemia disease, 24% had small, 16% had medium, and 12% had large ischemic lesions. Multifocal lesions were twice as common in the patients with sickle cell-thalassemia disease (20%) as in those with thalassemia intermedia (12.5%). Only in the patients with thalassemia intermedia did the frequency of brain damage increase with age. Moreover, brain damage inversely correlated with hemoglobin level in patients with thalassemia intermedia but not in those with sickle cell-thalassemia disease. Brain damage was more severe in patients with sickle cell-thalassemia disease who had more crises per year. CONCLUSION: This study suggests that patients with thalassemia intermedia and those with sickle cell-thalassemia disease may have asymptomatic brain damage. Our results suggest that MR imaging is useful in identifying patients at risk for stroke so that they can be treated with transfusional or pharmacologic therapy.
Authors: Elliott P Vichinsky; Lynne D Neumayr; Jeffrey I Gold; Michael W Weiner; Randall R Rule; Diana Truran; Jeffrey Kasten; Barry Eggleston; Karen Kesler; Lillian McMahon; Eugene P Orringer; Thomas Harrington; Karen Kalinyak; Laura M De Castro; Abdullah Kutlar; Cynthia J Rutherford; Cage Johnson; Joel David Bessman; Lanetta B Jordan; F Daniel Armstrong Journal: JAMA Date: 2010-05-12 Impact factor: 56.272
Authors: Michael R DeBaun; F Daniel Armstrong; Robert C McKinstry; Russell E Ware; Elliot Vichinsky; Fenella J Kirkham Journal: Blood Date: 2012-02-21 Impact factor: 22.113