What are the possible complications of severe combined immunodeficiency (SCID) and how can they be prevented?

Updated: Apr 28, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

Patients are at risk for infections from inadequate immune reconstitution from bone marrow transplantation (BMT) or enzyme replacement. Opportunistic infections usually follow more common infections. P jiroveci and fungal pneumonias cause death in classic cases. CMV, VZV, and HSV infections typically occur in infants who have already had treatable infections. Neurologic compromise from polio and other enteroviruses precludes stem cell reconstitution.

Ensure that the child does not receive any live virus vaccines until after BMT engraftment, especially polio or bacille Calmette-Guérin (BCG) vaccine. Vaccinating children with SCID is not only futile, because they cannot make antibody, but also dangerous, because they can develop disease (eg, poliomyelitis) from attenuated viruses and may even die after exposure to these vaccines.

Graft failure with BMT and posttransplant GVHD are well recognized, although both have decreased with improved BMT preparatory techniques. GVHD) may ensue if the blood products given before BMT are not depleted of white blood cells by filtration or irradiation. Ensure that all blood products are also negative for CMV to avoid systemic CMV disease.

Gene therapy has been associated with virus-induced malignancies. Cancer, usually non-Hodgkin lymphoma, is seen in patients with cartilage-hair hypoplasia who survive beyond early childhood.


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