Faculty Research 1980 - 1989

Title

Marrow transplantation in the treatment of a murine heritable hemolytic anemia.

Document Type

Article

Publication Date

5-15-1989

Keywords

Anemia, Hemolytic, Congenital, Animals, Bone Marrow, Bone Marrow Transplantation, Erythrocyte Count, Glucose-6-Phosphate Isomerase, Hematopoietic Stem Cells, Male, Mice, Mice, Mutant Strains, Phenotype, Postoperative Complications, Radiation Chimera

Publisher

American Society of Hematology

JAX Source

Blood 1989 May 15; 73(7):2014-7.

Abstract

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.

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