Several transgenic murine models for sickle cell anemia have been developed
that closely reproduce the biochemical and physiological disorders in the
human disease. A comprehensive characterization Is described of hematologic
parameters of mature red blood cells, reticulocytes, and red cell precurso
rs in the bone marrow and spleen of a murine sickle cell model in which ery
throid cells expressed exclusively human alpha, gamma, and beta (s) globin.
Red cell survival was dramatically decreased in these anemic animals, part
ially compensated by considerable enhancement in erythropoietic activity. A
s in humans, these murine sickle cells contain a subpopulation of phosphati
dylserine-exposing cells that may play a role in their premature removal. C
ontinuous in vivo generation of this phosphatidylserine-exposing subset may
have a significant impact on the pathophysiology of sickle cell disease. (
C) 2001 by The American Society of Hematology.