What are the exacerbating factors in severe combined immunodeficiency (SCID)?

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

A number of exacerbating factors may be present in patients with SCID. In most cases, the presence of maternal T cells is asymptomatic; however, approximately 30-40% of infants with SCID develop mild changes, such as erythema with skin T-cell infiltration, eosinophilia, elevated liver enzyme levels, and periportal T-cell infiltration. However, no cases of maternal graft-versus-host disease (GVHD) fatality have been reported.

GVHD can occur after engraftment of allogeneic immunocompetent lymphocytes because of incompatible bone marrow grafts or transfusion of blood products. Signs and symptoms include necrotizing erythroderma, gut mucosal abrasion, and biliary epithelium destruction.

In the past, when infants were routinely immunized with vaccinia virus, many infants with SCID died of vaccinia gangrenosa or progressive vaccinia. The bacille Calmette-Guérin (BCG) vaccine is still widely used in many countries; it can lead to a disseminated, fatal infection, revealing SCID. [32] Live vaccines, such as BCG and varicella vaccines, must not be administered to patients with SCID.


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