[Treatment of severe combined immunodeficiency with bone marrow or foetal liver transplants (author's transl)].
Nine children with severe combined immunodeficiency were treated in Lyon and Saint-Etienne. Two children had adenosine deaminase deficiency; another presented with a "bare lymphocyte syndrome", i.e. combined immunodeficiency associated with lack of expression of HLA-A and -B antigens on lymphocytes. Five children were treated with a transplant of genotypically HLA-identical bone marrow. The other 4 children received a transplant of foetal liver and foetal thymus from the same donor, as no compatible bone marrow donor was available. Seven of the 9 patients are still alive, reconstitution of the immune system being complete in 4 and incomplete in 3, and these results have been maintained throughout the years following transplantation. The only 2 failures were due to severe, widespread BCG infection, which had developed and persisted for several months before treatment. The measures now available for the treatment of infants with severe combined immunodeficiency are frequently successful in a disease previously lethal within a few months. They also contribute to our knowledge of lymphocyte differentiation and functions.
D000243:Adenosine Deaminase / Q000172:deficiency; D001853:Bone Marrow / Q000196:embryology; D016026:Bone Marrow Transplantation; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D008099:Liver / Q000196:embryology; D016031:Liver Transplantation; D008297:Male; D013950:Thymus Gland / Q000196:embryology / Q000637:transplantation; D014184:Transplantation, Homologous
D006680:HLA Antigens; D000243:Adenosine Deaminase
Traitement des déficits immunitaires combinés sévères par greffe de moelle osseuse ou de foie foetal.