What is included in the long-term monitoring of 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

Ensure regular follow-up visits to monitor the immune system, with specialist physicians monitoring the SCID patient. Isolation to avoid transmission of infection is required. Usually, contacts are restricted to immediate family members and friends whose risks for infection can be monitored. Visits to doctors’ offices and hospitals must be orchestrated carefully to avoid exposure to infection.

Although allogeneic hematopoietic stem cell transplantation (HCST) is curative for SCID, the long-term outcome in a 90-patient cohort followed for 2-34 years showed that almost half experienced 1 or more significant clinical events, including persistent chronic GVHD, autoimmune and inflammatory manifestations, opportunistic and nonopportunistic infections, and a requirement for nutritional support. [59] These late-onset complications suggest the need for prevention and careful follow-up.


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