Research Identifies Need for Improved Understanding of Sickle Cell Disease in... (cantly associated with ischemic lesions....)

Research Identifies Need for Improved Understanding of Sickle Cell Disease in Adulthood

Date:12/8/2007

cantly associated with ischemic lesions. Adjusting for age and gender, hippocampal volume (the size of the hippocampus, the part of the brain related to memory) in patients was 541.1 L less than controls and cerebrospinal fluid volume in patients was 16 mL greater than controls. These differences are significant.

The level of hemoglobin (the oxygen-carrying molecule in the blood) was an independent predictor of verbal IQ, Performance IQ, Processing Speed Index, math, and executive function. Patients with lower hemoglobin scores had lower IQ scores, and ischemic lesions alone did not account for the extent of the neuropsychological impairment observed. Hemoglobin levels, age, and hippocampal volume were predictive of the neuropsychological dysfunction, suggesting a link between reduced oxygenation, neuronal loss, and cognitive impairment. These data support the importance of future trials on transfusion and improvement of neuropsychological function.

-- Sequential deferiprone-deferoxamine superior to deferiprone monotherapy, the current "gold standard" in patients with thalassemia major [Abstract #575]

Aurelio Maggio, MD, Hospital 'V. Cervello', Palermo, Italy

This five-year, multicenter, randomized trial evaluated two chelating regimens in 140 patients with thalassemia major, a genetic disorder characterized by the underproduction of hemoglobin, the molecule in red blood cells that carries oxygen to all tissues. Chelating agents remove excess iron from the body, a common problem in red blood cell disorders because of the associated blood transfusions. Excess iron can lead to organ damage and heart failure. This trial evaluated deferiprone monotherapy compared with sequential treatment with deferiprone for four days followed by deferoxamine for three days. While deferiprone is an oral drug, deferoxamine is administered via subcutaneous infusion.

The study found that long-term use of sequential deferiprone-deferoxamine treatm
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