Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia

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.