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MHC Deficiency

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Topic updated on 12/24/15 3:06am

MHC class I deficiency
  • Introduction 
    • bare lymphocyte syndrome type I
      • mutation in TAP1 molecule
        • cannot transport peptides into ER for loading onto MHC I
      • absent CD8+
  • Presentation
    • recurring bacterial infections
    • can control some viral infections with NK cells
  • Evaluation
    • normal CD4+ counts
    • decreased CD8+ counts
    • normal B cell counts
MHC class II deficiency
  • Introduction
    • bare lymphocyte syndrome II
  • Presentation
    • mimics mild form of SCID
      • main difference is that lymphocytes do respond to mitogens
    • common symptoms
      • failure to thrive, diarrhea, infections of respiratory tract
    • do not develop graft-versus-host rejection
      • MHC II required
  • Evaluation
    • CD4+ deficiency
    • normal B cells


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