DEFECTS IN LEUKOCYTE FXN/COMPLEMENT SYS

Question 1

defective leukocyte adhesion because of mutations in the beta chain of CDII/CD18 integrins

Answer 1

leukocyte adhesion deficiency I

Question 2

defective leukocyte adhesion because of mutations in fucosyl transferase required for synthesis of sialylated oligosaccharide (receptor for selectins)

Answer 2

leukocyte adhesion deficiency II

Question 3

decreased leukocyte functions because of mutations affecting protein involved in lysosomal membrane traffic

Answer 3

Chediak-Higashi syndrome

Question 4

decreased oxidative burst

Answer 4

chronic granulomatous disease

Question 5

phagocyte oxidase (membrane component)

Answer 5

x-linked

Question 6

phagocyte oxidase (cytoplasmic components)

Answer 6

autosomal recessive

Question 7

decreased microbial killing because of defective MPO-H2O2 system

Answer 7

myeloperoxidase deficiency

Question 8

defective classical pathway activation; results in reduced resistance to infection and reduced clearance of immune complexes

Answer 8

C2,C4 deficiency

Question 9

defects in all complement functions

Answer 9

C3 deficiency

Question 10

excessive complement activation; clinical syndromes include angioedema, paroxysmal hemoglobinuria and others

Answer 10

deficiency of complement regulatory proteins