Transplantation in severe combined immunodeficiency disease with hl-a identical bone marrow.
The immunologic reconstitution of 7 patients with severe combined immunodeficiency disease was attempted with bone marrow transplantation from histoidentical donors. Four patients were successfully reconstituted and discharged from the hospital. Two patients died with sepsis. One patient died from a preexisting neurologic disease. All the patients who have been successfully reconstituted have had some degree of graft-vs-host disease. A dose of 50 x 10(6) nucleated bone marrow cells per kg seems necessary for successful engraftment. The use of density gradient separation had no advantage over whole, unfractionated bone marrow.
D000818:Animals; D000906:Antibodies; D000917:Antibody Formation; D001787:Blood Group Incompatibility; D001854:Bone Marrow Cells; D016026:Bone Marrow Transplantation; D002175:Candida / Q000276:immunology; D002180:Candidiasis, Oral / Q000209:etiology; D002469:Cell Separation; D002499:Centrifugation, Density Gradient; D003967:Diarrhea / Q000209:etiology; D005243:Feces / Q000382:microbiology; D005260:Female; D006041:Goats / Q000276:immunology; D006087:Graft vs Host Reaction; D006648:Histocompatibility; D006801:Humans; D007122:Immunoelectrophoresis; D007075:Immunoglobulin M / Q000032:analysis; D007136:Immunoglobulins / Q000032:analysis; D007153:Immunologic Deficiency Syndromes / Q000628:therapy*; D007223:Infant; D007621:Karyotyping; D008213:Lymphocyte Activation; D012486:Salmonella typhimurium / Q000302:isolation & purification; D012882:Skin Tests; D013154:Spleen / Q000473:pathology; D013950:Thymus Gland / Q000002:abnormalities
D000906:Antibodies; D007075:Immunoglobulin M; D007136:Immunoglobulins