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Blood, 1 September 2001, Vol. 98, No. 5, pp. 1577-1584
RED CELLS
Short survival of phosphatidylserine-exposing red blood cells
in murine sickle cell anemia
Kitty de Jong,
Renee K. Emerson,
James Butler,
Jacob Bastacky,
Narla Mohandas, and
Frans A. Kuypers
From the Children's Hospital Oakland Research
Institute and the Lawrence Berkeley National Laboratory, CA.
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 precursors in the bone marrow and spleen of
a murine sickle cell model in which erythroid cells expressed
exclusively human  ,  , and  S globin. Red cell
survival was dramatically decreased in these anemic animals, partially
compensated by considerable enhancement in erythropoietic
activity. As in humans, these murine sickle cells contain a
subpopulation of phosphatidylserine-exposing cells that may play a
role in their premature removal. Continuous in vivo generation of this
phosphatidylserine-exposing subset may have a significant impact on the
pathophysiology of sickle cell disease.
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