Gr. Honig et al., FETAL HEMOGLOBIN EXPRESSION IN TRANSPLANT RECIPIENTS OF PLACENTAL BLOOD HEMATOPOIETIC PROGENITOR CELLS, Pediatric research, 37(4), 1995, pp. 432-436
Patients who achieved bone marrow engraftment of cord blood-derived pr
ogenitor cells provided an opportunity to examine the expression of fe
tal Hb by neonatal hematopoietic progenitors in a postneonatal host. C
ord blood cells from histocompatible siblings were successfully transp
lanted in two children with the Fanconi anemia syndrome. One of the tr
ansplant donors had heterocellular hereditary persistence of fetal Hb,
apparently due to gamma-globin gene triplication; the other donor was
hematologically normal. The (G) gamma/(A) gamma ratio of the patient
who received his transplant from the donor with hereditary persistence
of fetal Hb was markedly elevated, similar to that of the transplant
donor's cord blood, and this ratio remained elevated in subsequent mon
ths. In the other child, the (G) gamma/(A) gamma ratio immediately aft
er her transplant was typical of the normal newborn, and over the next
several months it reverted to the adult pattern. Globin synthesis stu
dies performed shortly after engraftment demonstrated ratios of fetal
Hb/adult Hb synthesis in both patients that were typical of those of n
ormal newborns. Over the next several months, both patients converted
to the adult pattern. Fetal Hb to adult Hb switching in these patients
seemed to follow a temporal sequence intrinsic to the transplanted ne
onatal progenitor cells, without discernible influence of postneonatal
environmental factors. The program for Hb switching seems to be an in
herent feature of neonatal hematopoietic progenitor cells.