Immunology - Dx + Mx of Immunodeficiencies Flashcards
What are some consequences of phagocyte deficiencies?
- Recurrent deep bacterial infections
- Recurrent fungal infections
How are phagocyte deficiencies diagnosed?
NBT:
- Dye that changes colour from yellow to blue following interaction with hydrogen peroxide
DHR Flow Cytometry:
- DHR oxidised to rhodamine (strongly fluorescent), following interaction with hydrogen peroxide
How are phagocyte deficiencies treated?
- Aggressive management of infection
- Infection prophylaxis (Abx = Septrin, antifungals = itraconazole)
Definitive therapy:
- Haematopoeitic stem cell transplantation (replaces defective population)
What are some consequences of complement deficiencies?
- Increased susceptibilty to encapsulated bacterial infections
How are complement deficiencies diagnosed?
- Complement levels
- CH50 + AH50 tests
How are complement deficiencies treated?
- Vaccination
- Prophylactic Abx
- High levels of suspicion + early + aggressive treatment
- Screen family members
What happpens to C3, C4, CH50 + AP50 levels in the following conditions:
- C1q deficiency
- Factor B deficiency
- C9 deficiency
- SLE
Clq deficency
- C3 + C4 = +
- CH50 = decreased
- AP50 = +
Factor B deficiency
- C3 + C4 = +
- CH50 = +
- AP50 = decreased
C9 deficiency
- C3 + C4 = +
- CH50 + AP50 = decreased
SLE
- C3 = +/decreased
- C4 = decreased
- CH50 = +/decreased
- AP50 = +
How are lymphocyte deficiencies diagnosed?
- WCC
- Lymphocyte subsets
- Serum immunoglobulins (if CD4 deficient as IgG is surrogate marker for function) + protein electrophoresis
- Functional tests
- HIV
How are T cell deficiencies treated?
- Infection prophylaxis + treatment
- Ig replacement
- Haematopoeitic stem cell transplantation
- Gene therapy
- Thymic transplantation (Di George syndome)
How are B cell deficiencies treated?
- Aggressive treatment of infection
- Ig replacement every 3 weeks (pooled plasma containing diverse IgG)
- BMT
- Immunisation in Selective IgA deficiency
