Allergy & Immunology Flashcards
What are contraindications to vaccines?
- Anaphylaxis to a vaccine component
- Severe asthma and LAIV
- Intussusception and rotavirus
- Hx of GBS within 6wk of vacation
- Immunocompromised persons and live vaccines (including those with strong suspicion of immunodeficiency in their families)
- Immunosuppressive therapy
- Pregnancy and live vaccines
- Active TB and MMR /MMRV/Varicella (live vaccines)
What are five types of B cell (humoral) immunodeficiency?
- X-linked agammaglobulinemia (onset w/in 2y with recurrent sinopulmonary infections and abscent lymph nodes and tonsils)
- Common variable immune deficiency (CVID) - (onset >10y with Sinupulmonary infections, autoimmune, granulomatous disease, GI issues, malignancy)
- Selective IgA deficiency
- Specific antibody deficiency
- IgG subclass deficiency
What are the 10 warning signs of primary immunodeficiency?
- 4+AOM in 1y
- 2+ serious sinus infection in 1y
- 2+ pneumonias in 1y
- 2+ months of ABx with little effect
- 2+ deep-seated infections including septicemia
- FTT
- Recurrent, deep skin or organ abscesses
- Persistent thrush in mouth or fungal infection on skin
- Need for IV ABx to clear infections
- FHx of history of PI
Others:
- Autoimmunity (cytopenias, endocrinopathies, GI disturbance)
- Difficult to treat dermatitis despite treatment adherence
- EBV-associated malignancies
- A severe infection (e.g. invasive pneumococcal infection - 25% will have primary immunodeficiency)
What is the differential for immunodeficiency?
What investigations should you order if you suspect a humoral immunity defect?
Humoral Immunity:
• CBC and differential
• Serum immunoglobulins (how do you order this?)
• Serum specific antibody titres (diptheria, tetanus, can consider pneumococcal, MMR)
• Flow cytometry to identify B-cell markers and numbers
Advanced humoral testing:
• Flow cytometry to determine B-cell subsets (i.e. naïve vs switched memory)
• In vitro immunoglobulin production to mitogens
What investigations should you order if you suspect a T-cell immunity defect?
T-cell defects
• Newborn Screening
• CBC and differential
• Flow cytometry (CD4, CD8, NK cells)
• In vitro proliferation to mitogens (i.e. pokeweed, concanavalin A)
• Cutaneous delayed hypersensitivity
Advanced T-cell tests
• T-cell subset analysis (naïve, memory, activated)
What investigations should you order if you suspect a Phagocytic defect?
- CBC and differential
- Dihydrorhodamine testing (or NBT)
- Flow cytometry for adhesion molecules
What investigations should you order if you suspect a Complement defect?
- CH50 assay (classical complement pathway)
- AH50 assay (alternative complement pathway) • MBL (mannose binding lectin pathway)
- C3/C4
What pathogens are most associated with chronic granulomatous disorder?
- Pathogens: Catalase positive (staph, aspergillus, nocardia, serratia marcescens, burkholderia cepacia, salmonella)
- Infections: Recurrent bacterial and fungal infections - things like adenitis, empyema/PNA, abscess, OM, sepsis, granulomas
- Can have IBD
What are the clinical features of Hyper IgE Syndrome?
- Recurrent abscesses in lung, skin - these present as “cold boils” because they’re not your usual hot tender abscesses. Caused by staph and can have pneumatoceles.
- Can have mucocutaneous candidiasis
- Eczema, papulopustular lesions
- Facial features - deep set eyes, prominent forehead, broad nasal bridge, bulbous nose, coarse skin
- Delayed shedding of teeth and fractures!
What are the different treatments for CGD, HyperIgG and LAD?
- CGD - abx (TMP-SMC), and itraconazole (fluco not good for aspergillus), anti-inflammatories
- HyperIgG - anti-staph prophylaxis with Septra
- LAD - abx, HSCT
What are the clinical features of X-linked agammaglobulinemia?
- Present with recurrent bacterial sinopulmonary infections
- Typically start in the first 2 years of life
- Most common infectious organisms – Streptococcus pneumoniae, Haemophilus influezae
- Other microorgansms of concern – ECHO virus, Helicobacter, Campylobacter, Pseudomonas, Ureaplasma, Mycoplasma
- Absence of LN on exam!
What is the management of X-linked aggamaglobulinemia?
- Repalcement immunoglobulins
- Antimicrobial prophylaxis
- Recurrent monitoring of pulmonary status - These patients are at risk of bronchiectasia
- Consider lung transplant in patients with agammaglobulinemia and life-threatening chronic lung disease
- Can present with enteroviral meningitis – requires treatment with IV Ig
What is the definition of CVID (note: universal definition does not exist)
- Low IgG and low IgA/M with poor response to vaccines
- Exclude other causes of humoral immunodeficiency (note: B cells can be normal)
What is the triad of Wiskott-Aldrich Syndrome?
- Eczema
- Thrombocytopenia (uniformly small platelets)
- Recurrent infections (sinopulmonary and viral)