1. Adaptive
   1. Humoral
      1. B-cells (Ig, CD40, 50)
         1. X-linked Agammaglobunemia (Bruton's)
            1. Pt: Boys, B-cells @6mo Sinopulmonary infxns
            2. normal CBC
            3. QIg: no IgA, no IgG, no IgM
            4. Flow: no B cells
            5. BTK Gene
            6. Schedule IgG
            7. BM Transplant?
         2. Common Variable Immunodeficiency (CVID)
            1. mild XLA
            2. presents during teenage years
            3. normal CBC
            4. QIg: low 2/3 Ig's
            5. Schedule IgG?
         3. IgA Deficiency
            1. poor mucosal defenses
            2. Sinopulmonary infxns Bouts of GI bugs
            3. ASx - until analphylaxis after blood transfusion
            4. normal CBC
            5. QIg: low IgA, more IgG, IgM
            6. no Tx, f/u anaphylaxis
         4. Hyper IgM
            1. cannot convert IgM to IgG
            2. non-specific immunodef
            3. normal CBC
            4. QIg: high IgM, low IgG, IgA
            5. no Tx
   2. Combination
      1. Wiskott-Aldrich
         1. X-linked: boys
            1. atopic dermatitis low platelets normal infxns
            2. CBC: low WBCs, low PLTs
            3. QIg: high IgM, IgG
            4. BM Tx
      2. Ataxia Telangiectasia
         1. immunodef
            1. DNA repair mech leukemia lymphoma
      3. SCID
         1. ADA def
            1. no B's, no T's
            2. immediate immunodef
            3. CBC: low WBCs
            4. QIg: no IgM, IgG, IgA
            5. Bubble Baby
            6. PPx: TMP-SMX
            7. BM Tx
   3. Cellular
      1. T-cells (CD4, CD8)
         1. DiGeorge
            1. 22q11.2 del 3rd pharyngeal pouch
            2. CATCH-22 wide-spaced eyes low set ears small face
            3. absent thymic shadow
            4. infxns w/ Fungi + PCP
            5. Dx: Clx
            6. CBC: low lymphocytes
            7. PPx: TMP-SMX
            8. IVIg
            9. Thymic transplant
            10. f/u low Ca
         2. Acquired HIV
2. Innate
   1. Phagocytosis
      1. Macrophages
         1. Chronic Granulomatous Disease
            1. no respiratory burst but can phagocytize
            2. susceptible to catalase-positive orgs
            3. Staph abscesses
            4. Dx: Nitroblue test
            5. CBC: hi WBCs
            6. QIg: high IgM, IgG
            7. BM Tx
         2. Leukocyte Adhesion Deficiency
            1. WBCs can't leave the bloodstream
            2. delayed separation of the cord
            3. incr fever incr WBC no pus
            4. Dx: Clx
            5. BMT
         3. Chediak-Higashi
            1. autosomal recessive
            2. giant granules in neutrophils
            3. assoc w/ albinism neuropathy neutropenia
      2. NK
   2. Complement
      1. C1-Esterase
         1. spontaneous angioedema
         2. FFP
      2. C5-C9 Mac Attack
         1. if infected with Neisseria
3. Work-up
   1. CBC + Diff
   2. Quant IG levels
      1. IgA
      2. IgG
      3. IgM