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43 Cards in this Set
- Front
- Back
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What % of all primary immunodef is Adaptive?
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80%
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What percent are Bcell?
What percent are Tcell or combined B/Tcell? |
Bcell = 50%
T or combined = 30% |
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When is the onset of Bcell defects?
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4-6 mo of age (when maternal Ab wears off)
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When is the onset of Tcell defects?
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Usually before 6 months old
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What does a Bcell defect present with?
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-Recurrent sinupulmonary infections
-Chronic GI infections showing malabsorption and poor growth |
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What types of infections will babies with Bcell defects get?
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Bacterial - especially encapsulated, and viral
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What does a Tcell defect present with?
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Poor growth, failure to thrive, and chronic diarrhea
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What types of infections will Tcell-defective patients get?
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Viral, fungal, and opportunistic infections
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What percentage of all PID are innate?
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20%
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What are the 4 types of innate immune system deficiencies?
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1. Neutrophil defect
2. Macrophage defect 3. NK cell defect 4. Complement defect |
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When is the onset of most of the innate deficiencies?
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In infancy or childhood
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How does an neutrophil deficiency present?
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With common pathogens causing severe infections
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What are the typical agents of infection in neutrophil deficiency?
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Bacterial - Staph, pseudomonas
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What are the common presentations in macrophage defects?
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-Recurrent fevers
-Disseminated mycobacterial infections |
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What is the common disease associated with macrophage defects?
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Mycobacterial disease
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What is the typical presentation of a NK cell defect?
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Disseminated viral infections or Cancer
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What is the common presentation of a Complement defect?
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Rheumatoid disorders like lupus or scleroderma
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What infection is typically seen in complement defects?
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Neisseria
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What type of disorders will exhibit an abnormal immune response that never really gets up and goes?
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SCIDs
Digeorge syndrome |
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What type of disorders will exhibit an abnormal immune response that sort of gets up, but then wavers up and down?
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Autoinflammatory disorders
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What type of disorders will exhibit an abnormal immune response that gets up and goes and goes and goes?
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Immune dysregulation syndromes
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What is the normal number of otitis media infections per year?
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6-8
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What is considered an abnormal number of sinus infections per year?
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>2
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How many episodes of pneumonia per year would be abnormal?
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>2
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When is thrush abnormal?
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Over the age of 1 yr old
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Why is thrush common in children under 1 yrs old?
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-Nipple colonization
-Freq use of antibiotics for ear infections decreases the normal protection against thrush. |
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What do the majority of PIDs have some degree of?
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GI complications
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What are 3 GI infections that are suggestive of PID?
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-Giardia
-Cryptosporidium -Recurrant/refractory C.diff |
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If there are recurrent sinupulmonary infections
poor growth and diarrhea... think: |
Humoral (Bcell) defect
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What bacteria will cause infections in Bcell defects?
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Encapsulated
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3 types of encapsulated bacteria:
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-Haemophilus
-Pneumococci -Streps |
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If you see abcesses, open sores without pus, or poor wound healing, oral ulcers, recurrent pneumonias, think:
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Neutrophil defect
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Bacteria, fungi, and parasites that will cause infections in neutrophil defects:
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SPSK CAN
Staph aureus Pseudomonas Serratia, Klebsiella Candida Aspergillus Nocardia |
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Failure to thrive
Chronic diarrhea Opportunistic infections Polyendocrinopathy Thrush... THINK: |
Tcell or combined T/Bcell defect
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What will the family history show in Tcell or combined T/B cell defect?
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History of early childhood deaths
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You suspect a humoral deficiency; what lab test will you order?
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HISS panel
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What is a HISS panel?
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Humoral Immune Status Survey
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What does a HISS panel give you?
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Antibody levels and vaccine titers
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You suspect a Tcell or combined immune defect; what would you order?
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-Lymphocyte subsets
-HISS panel -Skin testing |
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You suspect a phagocyte defect; what would you order?
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1. CBC with diff
2. NBT/oxidative burst test |
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You suspect a complement defect; what would you order?
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Total Hemolytic Complement assay
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What does a Total Hemolytic Complement assay measure?
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Both the classical and alternative pathways
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Why is flow cytometry an important assay?
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Because a CBC only gives the basic numbers and is not complete.
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