Chediak-Higashi syndrome: reversal of increased susceptibility to infection by bone marrow transplantation.
Transplantation of normal bone marrow to mice with the Chediak-Higashi syndrome (CHS) resulted in normal granulopoiesis and a reversal of their increased susceptibility to challenge with intravenous Candida albicans. These findings suggest that (1) the leukocyte defect in CHS can be reversed by marrow transplantation and (2) the mechanism for increased susceptibility to infection in these animals is due to a bone-marrow-derived cellular defect. Because of similarities between murine and human CHS, bone marrow transplantation might be considered as a mode of therapy in selected cases of the human disease.
D000818:Animals; D001854:Bone Marrow Cells; D016026:Bone Marrow Transplantation; D002177:Candidiasis / Q000150:complications; D002609:Chediak-Higashi Syndrome / Q000150:complications / Q000401:mortality / Q000628:therapy*; D005260:Female; D007958:Leukocyte Count; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D011828:Radiation Chimera; D014184:Transplantation, Homologous