Primary Innate Immunodeficiency Flashcards
How are Primary Immunodeficiencies acquired?
Genetics
What are the Primary Innate Immunodeficiencies - leukocyte disorders?
Leukocyte Adhesion Deficiency 1 and 2
Chediak Higashi Syndrome
Chronic Granulomatous Disease
What are the Primary Innate Immunodeficiencies - leukocyte disorders?
Leukocyte Adhesion Deficiency 1 and 2
Chediak Higashi Syndrome
Chronic Granulomatous Disease
Leukocyte disorders cause an issue in what cell type mediated immune response?
Neutrophils
Leukocyte Adhesion Deficiency 1 has a defective?
Beta2 Integrin
Defective Beta2 integrin
Leukocyte Adhesion Deficiency 1
Leukocyte Adhesion Deficiency 2 has a lack of?
Selectin ligands (NO fucosyl transferase)
Lack of selectin ligands (no fucosyl transferase)
Leukocyte Adhesion Deficiency 2
Leukocyte Adhesion Deficiencies and what they lack?
LAD 1 - Defective Beta2 Integrin
LAD 2 - Lack selectin ligands (no fucosyl transferase)
How is Chediak Higashi Syndrome inherited?
Autosomal Recessive
Chediak Higashi Syndrome has a failure of?
Failure of phagolysosomal fusion
Failure of phagolysosomal fusion
Chediak Higashi Syndrome
Symptoms of Chediak Higashi Syndrome?
Albinism - grey hair
Easy bruising and bleeding
Albinism with grey hair and easy bruising is what primary innate immunodeficiency?
Chediak Higashi Syndrome
- failure of phagolysosomal fusion
What will be seen morphologically with Chediak Higashi Syndrome?
Giant granules
- Failure of phagolysosomal fusion
Chronic Granulomatous Disease has failure of?
Failure of superoxide production within phagocytes
Failure of superoxide production within phagocytes
Chronic Granulomatous Disease
Chronic Granulomatous Disease involves no superoxide production in phagocytes. What happens?
Macrophages “wall off” infections
Macrophages “walling off” infections is seen with?
Chronic Granulomatous Disease
Leukocyte disorders of primary immunodeficiency are at risk for what type of infections?
Bacterial - s. aureus, strep/enterococcus
Primary innate immunodeficiency complement disorder?
Membrane Attack Complex Deficiency
Primary innate immunodeficiency complement disorder?
Membrane Attack Complex Deficiency
What parts of the MAC complex can be affected with the primary disorder?
C5 - C9
If there is no MAC, what will no occur?
NO lysis of microbe
Membrane Attack Complex Deficiencies are at risk for what type of infections?
Neisseria
– meningitis
Is Hereditary Angeioedema an immunodeficiency?
NOOO
How is Hereditary Angioedema inherited?
Autosomal Dominant
What is deficient with Hereditary Angioedema?
C1 - inhibitor
What is unregulated with Hereditary Angioedema?
Kallikrein and complement pathways are unregulated
Symptoms of Hereditary Angioedema?
Edema at multiple sites
