Sickle cell anemia and transposition of the great vessels
R. L. Hudson, O. Castro, J. L. Spivak, C. Sampson and J. W. Downing
A child with homozygous sickle cell disease and transposition of the great
vessels had erythrocytosis associated with markedly increased plasma
erythropoietin activity. Her clinical course was complicated by neurologic
manifestations but not by recurrent sickle cell vasooculsive episodes. The
fetal hemoglobin level which had been greater than 25% during the first two
years of life gradually decreased to less than 10%. She died at 3 years of
age of congestive heart failure and severe anemia. The only sickle cell
painful crisis occurred during her terminal illness. It is likely that the
high levels of fetal hemorglobin decreased sickling and thus allowed
erythrocytosis to develop. Fetal hemoglobin may also have prevented
frequent vaso-occlusive events despite the high hematocrit level.
