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Sickle-Cell Anemia Associated with Tetralogy of Fallot

1965; Massachusetts Medical Society; Volume: 273; Issue: 20 Linguagem: Inglês

10.1056/nejm196511112732005

1533-4406

Howard A. Pearson, Gerold L. Schiebler, L. Jerome Krovetz, Thomas D. Bartley, Jesús David,

Blood groups and transfusion

THE fundamental defect of sickle-cell disease is a mutation that leads to substitution of a valine for a glutamic acid residue in the beta chains of hemoglobin.1 When red cells containing the chemically altered hemoglobin S are deoxygenated, elongated hemoglobin tactoids form, resulting in the distorted sickle cell. Sickled red cells increase blood viscosity and obstruct small blood vessels mechanically. A straightforward relation between oxygen tension and hemoglobin S forms the basis of most theories of the pathogenesis of sickle-cell disease.2 This report describes a unique patient who had both homozygous hemoglobin S and cyanotic congenital heart diseases. Our studies . . .