Primary → in the context of hereditary diseases. More in children
Secondary → in the context of diseases, infections (eg. HIV), treatments
Mechanism of various immune deficiencies
- Deficits in Humural immunity (Th2, B Lymphocytes)
- Pyogenic bacteria
- Parasites
- Deficits in Cellular immunity (Th1)
- intracellular pathogens (mycobacteria, fungi, protozoa)
- Deficits in Complement
- C3 (alternative pathway) important to clear immune complexes → more type III hypersensitivity
- C1, C2, C4 (classic pathway) → more autoimmune diseases (eg. SLE)
- Terminal pathway makes membrane attack complex → important to clear encapsulated organisms → streptococcus pneumoniae, haemophilus influenzae type B, neisseria meningitidis
- Deficits in phagocytosis
- Pyogenic bacteria and fungal infections
Primary Immune Deficiencies
Primary Imune Deficiencies predisposing to HPV
!Epidermodysplasia verruciformis, tree man syndrome or Lewandowsky-Lutz dysplasia!Whim syndromeAutosomal recessive Hyper-IgE SyndromeIdiopathic CD4 lymphopeniaDeficiencies in GATA2Netherton syndromeWILD syndromePrimary immunodeficiency associated with neonatal erythrodermia
Omenn syndrome