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Pathology

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Three general pathogenetic mechanisms may result in SCID:

  • T- and/or B - cell stem cell defects - the most common. The thymus is arrested early in development with the result that lymphoid tissue is virtually absent from the lymph nodes, spleen, tonsil and appendix. Opportunistic infections occur readily, often leading to death.

  • The thymic epithelium may be unable to induce normal T cell maturation and development.

  • defective B cell function occurs secondary to defective T cell help or aberrant T cell suppression

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