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