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Immunodeficiency (Immunodeficiency (Adaptive
Cell: T Cell
CD markers:…
Immunodeficiency
Immunodeficiency
Adaptive
Cell: T Cell
- CD markers: 4,8, single digits, teens
Humoral: B cells
- CD markers: 40, 50, and higher
- Ig
Innate
Cellular: NK, MPs
Humoral: Complement
Pt: Recurrent infections > 6m :baby::skin-tone-2:
- exposure should make them immune
- severe infx w/ normal bugs :spider:
- unusual pathogens
- FFT w/ diarrhea :poop:
- won't see <6m 2/2 :pregnant_woman::skin-tone-2: mom's Ig
Dx: CBC w/ diff
- ± quantitative Ig
- disease specific tests
Tx: Disease specific
B/T Combined
LY
T Cell
B Cell
XLA (Bruton's)
X linked agammaglobulinemia
- Boys :boy::skin-tone-2:
- B cells :bee:
Pt: immunodef @ 6m, Earliest
- sinopulmonary infx :nose::skin-tone-3:
- recurrent, rare, unusual
Dx: CBC normal
- Quantitative immunoglobulin
- Flow cytometry = no B cells :bee:
- confirm: RTK gene
Tx: Schedule IgG
- ±BMT, might be too severe
CVID
Mild form of XLA
Pt: :man::skin-tone-2: Teen w/ sinopulmonary infx
Dx: CBC normal
Tx: ± Scheduled IgG
IgA def
Can't fight mucosal infections
Pt: ears, nose, throat, gut
- sinopulmonary infx :nose: + gastroenteritis 🐜
- Asx until blood transfusion :syringe: → anaphylaxis
Dx: CBC normal
Tx: nothing, avoid transfusions
- IM epi if anaphylaxis
- screen future blood, remove IgA
Hyper IgM
B cell deficiency :bee:
- cant convert IgM → IgG
- IgM is nonspecific
- IgG is memory/specific
Pt: nonspec immune deficiency
Dx: CBC normal
Tx: nothing
DiGeorge
22q11.2 del
- prob of 3rd pharyngeal pouch
Pt: wide spaced eyes ←:eye:→
- low set ears ↓:ear:↓
- absent thymic shadow
- small face
- Infx w/ fungi & PCP
Dx: Clinical
- CBC ↓ absolute lymphocyte count
Tx: TMP-SMX prophy
- IVIG : bridge to thymic transplant
f/u: no PT → hypocalcemia :glass_of_milk:
-
Wiscott Aldrich
X linked
Pt: atopic dermatitis
- ↓platelets
- normal infections
Dx: CBC ↓WBC
- ↓platelets
- Quant Ig
- compensatory ↑IgM, ↑IgG
Tx: BMT
Ataxia Telangectasia
Ataxia
Telangectasia
Immunodeficiency
DNA repair, Leukemia, Lymphoma
SCID
- mega AIDS w/o the virus
- def Adenosine deaminase
Pt: any exposure runs rampant, Immediate immunodef
Dx: CBC ↓WBC
Tx: Bubble baby
- sterile room
*±TMP-SMX
- BMT nec for survival
Phagocytosis
CGD
MP prob: no respiratory burst
- eat, cant kill catalase (+)
Pt: Staph abscesses
Dx: Nitro Blue
- Blue = fine
- CBC ↑WBC
- Quant Ig
Tx: BMT
LAD
WBC can't extravasate
Pt: ↑ :fire:fever
- ↑leukocytes
- absent pus
- delayed separation of cord
Tx: BMT
Chediak Hidashi
Autosomal rec disease
- Giant PMN in granules
- albiniam
- Neuropathy
- neutropenia
Complement
C1 esterase deficiency
- spontaneous, non-drug related angioedema
Tx: FFP
-
- Identify disease based on clinical presentation
- LY topic
- B Cell/T cell or Combo problem
- Phagocytosis problem
- Complement problem