Four newborn infants with Down syndrome and manifestations of neonatal
leukemia are described. One of the four was stillborn, two died short
ly after birth, and a fourth survived and all evidence of leukemia dis
appeared in the first month of life. Three of the four cases had hydro
ps fetalis, and a fourth was a macerated stillborn. Nine other similar
reported cases are reviewed. We conclude that neonatal leukemia in Do
wn syndrome is a form of leukemia that is usually transient, with spon
taneous recovery, but may be fatal at or around the time of birth with
manifestations of hydrops fetalis, hepatosplenomegaly, and/or progres
sive liver disease.