Immunology Flashcards
Keys for Primary Immunodeficiency
ABCDEF 4321
- Antibiotics IV
- aBscesses
- Candidiases
- aDverse reactions to vaccines
- Especially if FTT
- Family History
4 - ears
3 - ALC < 3
2 - sinus, pneumonia
1 - invasive infection (especially if vaccine preventable)
Examples of immunodeficiencies
Organisms for each immunodeficiency
Investigations for Immunodeficiency
Humoral Immunity - B cells
- absent response to vaccines
- sinupulmonary infections
- encapsulated bacteria
- autoimmunity and increased malignancy
XLA
- male + fam history
- absent lymph tissue
- 4-6 months (loss of maternal)
- absent IgG/IgA/IgM
CVID
- lymph tissue present
- age > 2
- absent IgG. normal/low IgA/IgM
Tx
- monitor for autoimmunity/malignancy
- replace IgG
- follow PFTs (risk of bronchiectasis)
- no live vaccines for XLA
Cellular Immunity - T Cells
SCID
- fungal
- FTT
- < 3 months of age
- absent thymus
- needs HSCT
WAS
- eczema + petechiae + immunodef
- eosinophilia and elevated IgE
- small/low platelets
- needs HSCT
AT
- ataxia as a toddler
- telangiectasias by 4-5 years
- elevated AFP
- abnormal DNA repair
- supportive care
- increased risk of lymphoma
DiGEORGE
- cardiac defects
- hypocalcemia
- psychiatric
- severe = SCID
- cleft palate
- dysmorphic features
Phagocytic Immunity
CGD
- catalase positive
- abscesses annd granulomas
- Crohn’s like bowel
- abnormal neutrophil oxidative burst
- needs HSCT
LAD
- delayed umbilical cord separation (> 4 weeks)
- no pus formation
- neutrophilia
- absent surface adhesion molecules (CD11 & CD8)
- needs HSCT
Catalase Positive Organisms
- staph aureus
- aspergillus
- nocardia
-serratia - burkholderia
- salmonella
Complement Deficiency
- encapsulated bacteria, recurrent severe infections
- defects in C1, C2, C4 - rheumatic disease, SLE like
- defects in C5-9 - disseminated neisseria, meningitis
- abnormal CH50 and AH50
- antibiotic prophylaxis and immunizations
- immunosuppression for autoimmune conditions
