Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency
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.
Key wordsBCG infection Severe combined immunodeficiency Omenn syndrome Bone marrow transplantation Hypertrophic non-obstructive cardiomyopathy
bone marrow transplantation
hypertrophic nonobstructive cardiomyopathy
severe combined immunodeficiency
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