Immune Deficiencies

Bruton's

  • no receptors on B cells, can't make antibodies

  • encapsulated bacteria
  • enterovirus enter through GI track

IgA Deficiency

CVID

  • rare disorders in one disorders with variable degrees of deficiency

  • immune dysregulation: autoimmune

DiGeorge

  • copula: develop pharynx

  • similar to end stage HIV
  • improve with age, may mature in other locations
  • cleft palate, mandible: 3rd/4th pharyngeal pouches

Hyper-IgE

CMCC

  • autoimmune T cell attack endocrine glands

SCID

  • loss of entire adaptive immune system

  • live in a bubble
  • low CD3, low Ig levels

  • can't do T cell count
  • do TRECs

ADA

Ataxia

Hyper-IgM

Wiskott

  • immunologic synapse: way T react to APC
  • low or normal IgG/IgM. Elevated IgE/A

  • bleeding/petechiae unique

Innate

LAD

  • neutrophils can't get to stump

Chediak Higashi

  • unique: oculocutaneous albinism
  • melanocyte granules can't be transferred

CGD

  • catalase breaks down H2O2
  • catalase - can't break down H2O2
  • Burkhoderia capacia