5. Case Presentation 2

Question 1

What are the 5 invasive strains of Neisseria Meningitides?

Answer 1

A, B, C, Y and W-135

Question 2

What is the tetravalent meningococcal vaccine introducing in order to gain immunity?

Answer 2

Polysaccharide (not protein) components of A, C, Y, and W-135

Question 3

Why would someone without a spleen be more susceptible to microbes with polysaccaride antigens?

Answer 3

The spleen is the location of the Marginal zone B1 cells that respond to polysaccaride antigens.

Question 4

Deficiency of terminal complement proteins increases risk especially to what kind of bacteria?

Answer 4

Encapsulated bactera

Question 5

What is the immunopathology of Hereditary Angioedema (HAE)?

Answer 5

C1 inhibitor is nonfunctional or underexpressed, leading to uncontrolled complement activation. As a result, C2 and C4 is chronically low.

Question 6

What is the name of the purified C1inh that can be given in cases of potentially lethal Hereditary Angioedema of the respiratory tract?

Answer 6

Berinet

Question 7

What is the immunopathology of Paroxysmal Nocturnal Hemoglobinuria?

What is the treatment?

Answer 7

Patients experience hemolysis of RBCs overnight, leading to hemoglobin in their urine in the morning. Pt’s RBCs and platelets have an increases sensitivity to complement lysis due to a defect in the Phosphatidylinositol Glycan Class A Gene (PIG-A) leading to a defect in the GPI anchor that is necessary for CD55 and CD59 presentation, which resists MAC.

EPO for blood loss, corticosteroids, and eculizumab.