Marrow transplantation in the treatment of a murine heritable hemolytic
anemia
JE Barker and EC McFarland-Starr
Jackson Laboratory, Bar Harbor, ME 04609.
Mice with hemolytic anemia, sphha/sphha, have extremely fragile RBCs with a
lifespan of approximately one day. Neither splenectomy nor simple
transplantation of normal marrow after lethal irradiation cures the anemia
but instead causes rapid deterioration and death of the mutant unless
additional prophylactic procedures are used. In this report, we show that
normal marrow transplantation preceded by sublethal irradiation increases
but does not normalize RBC count. The mutant RBCs but not all the WBCs are
replaced by donor cells. Splenectomy of the improved recipient causes a
dramatic decrease in RBC count, indicating that the mutant spleen is a site
of donor-origin erythropoiesis as well as of RBC destruction. Injections of
iron dextran did not improve RBC counts. Transplantation of primary
recipient marrow cells into a secondary host with a heritable stem cell
deficiency (W/Wv) corrects the defect caused by residence of the normal
cells in the sphha/sphha host. The original +/+ donor cells replace the
RBCs of the secondary host, and the RBC count is normalized. Results
indicate that the environment in the sphha/sphha host is detrimental to
normal (as well as mutant) erythroid cells but the restriction is not
transmitted.
Volume 73,
Issue 7,
pp. 2014-2017,
05/15/1989
Copyright © 1989 by The American Society of Hematology
