Faculty Research 1980 - 1989

Title

Bone marrow replacement in the treatment of hemolytic disease in mice.

Document Type

Article

Publication Date

1989

Keywords

Animal, Bone-Marrow: re, Bone-Marrow-Transplantation: mt, Colony-Forming-Units-Assay, Electrophoresis-Cellulose-Acetate, Gamma-Rays, Genotype, Hematologic-Tests, Hemoglobins: an, Mice, Mice-Inbred-C57BL, Spectrin: ip, Whole-Body-Irradiation, X-Rays

JAX Location

1828

JAX Source

Exp Hematol 1989 Nov; 17(10):1004-10.

Abstract

Bone marrow replacement therapy following whole-body x- or gamma-irradiation has until now proven to be of limited value in the treatment of individuals with hemolytic disease. The large doses of radiation required for destruction of defective erythropoietic tissues coupled with their resultant high mortality appears to limit its usefulness. Techniques have been developed by the authors to limit the extent of exposure and to improve survival following irradiation. These techniques include shielding of all parts of the body except the hind limbs, prophylactic use of antibiotics, and preparatory blood transfusion to suppress the development of indigenous defective erythrocytes. Using these combined techniques we were able to establish high rates of survival, successful engraftment, and long-term clinical improvement in mice with several hemolytic disorders emanating from hereditary defects in spectrin production and incorporation. Evidence is presented indicating that complete bone marrow replacement occurs even in nonirradiated portions of the erythron and that only donor type red blood cells appear in the circulation.

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