Neuropsychological Effects of Immunosuppressive Treatment in Subjects With Aplastic Anemia

Cyclosporine (CsA) is widely used in the treatment of aplastic anemia as well as to provide immunosuppression after transplantation. CsA has a spectrum of neurologic and more subtle, poorly investigated neuropsychological functional effects. In the NHLBI, a unique opportunity exists to evaluate changes in neuropsychological function following CsA in a large cohort of aplastic anemia patients accrued to NHLBI treatment protocols. With increased success in treating blood diseases with immunosuppressive therapy or stem cell transplantation that utilize CsA in the treatment regimen, patients are enjoying prolonged survival during which quality of life becomes an increasingly relevant concern. We therefore propose this natural history protocol designed to evaluate neuropsychological functioning before, during, and after CsA administration in severe aplastic anemia patients. The primary objective is to evaluate changes in attention, language, memory, spatial/motor and executive function domains following CsA therapy using well established neuropsychological tests including the Neuropsychological Assessment Battery (NAB) Screening Module, Revised Hopkins Verbal Learning Test, Revised Brief Visual-spatial Memory Test, Judgment of Line Orientation test, Grooved Pegboard, Trail Making Test and the two performance subsets in Wechsler Adult Intelligence Scale-III. IQ test by Wechsler Abbreviated Scale of Intelligence and depression/anxiety level will be screened once prior to formal testing. Measures will also be correlated with clinical status, standard CsA side effects, CsA drug levels and relevant biochemical lab values at each time point to control the testing accuracy and explore potential correlation.