Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency |
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| Authors: | R S Heyderman G Morgan R J Levinsky S Strobel |
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| Institution: | (1) Department of Immunology, Institute of Child Health, 30 Guildford Street, WC1N 1EH London, UK;(2) Hospital for Sick Children, Great Ormond Street, WC1N 3JH London, UK;(3) Present address: Department of Paediatrics, Quees Elizabeth the Queen Mother Wing, St. Mary's Hospital Medical School, South Wharf Road, W2 1NY London, UK |
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| Abstract: | We report successful bone marrow transplantation (BMT) in two patients with severe combined immunodeficiency (SCID), who had developed BCG infection following neonatal vaccination. Patient 1 had Omenn Syndrome, associated with hypertrophic nonobstructive cardiomyopathy. Patient 2 had SCID due to adenosine deaminase deficiency. This communication demonstrates that with appropriate anti-mycobacterial cover, immunological reconstitution together with full recovery from BCG infection can be achieved by BMT. As demonstrated by persistant negative Mantoux tests, specific cell-mediated immunity to BCG was not acquired following BMT. We suggest that these children may continue to be at risk from mycobacterial infection. |
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| Keywords: | BCG infection Severe combined immunodeficiency Omenn syndrome Bone marrow transplantation Hypertrophic non-obstructive cardiomyopathy |
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