B Cell Deficiencies

Question 1

Deficiency tyrosine kinase that block B cell maturation

Answer 1

Bruton X linked agammaglobulinemia (Ig replacement)

Question 2

Low Ig, no circulating B cells

Answer 2

Bruton X linked agammaglobulinemia (Ig replacement)

Question 3

Deficiency of CD40 L on activated T cell

Answer 3

X linked hyper IgM syndrome (Ig replacement)

Question 4

High serum titers of IgM, susceptible to extracellular bacteria

Answer 4

X linked hyper IgM syndrome (Ig replacement)

Question 5

Most common B cell deficiency

Answer 5

Selective IgA deficiency

Question 6

Repeated sinopulmonary and GI infxn

Answer 6

Selective IgA deficiency

Question 7

Ig decrease over time

Answer 7

Common variable immunodeficiency (antibiotics)

Question 8

Delayed onset of maturation of normal IgG

Answer 8

Transient agammaglobulinia (antibiotics)

Question 9

Spontaneous resolution, diagnosed in infants

Answer 9

Transient agammaglobulinemia (antibiotics)

Question 10

DiGeorge syndrome

Answer 10

Congenital thymic aplasia

Question 11

Only disease that is T cell specific; facial abnormalities, hypoparathryoidism, cardiac malformation

Answer 11

DiGeorge syndrome

Question 12

CATCH22 syndrome

Answer 12

Cardiac abnormalities, abnormal facies, thymic hypoplasia, cleft palate, hypocalcemia

Question 13

Defect in cystoskeletal glycoprotein

Answer 13

Wiskott adlrich

Question 14

Defective response in bacterial polysaccharide and depressed IgM , gradual loss of humoral and immune response, thrombocytopenia and ECZYMA

Answer 14

Wiskot aldrich

Question 15

Adenosine deaminase deficiency

Answer 15

Severe combined ID

Question 16

Prone to opportunistic infection, low levels of circulating lymphocytes, chronic diarrhea, skin and mout infections

Answer 16

Severe combined immunodeficiecy

Question 17

Deficiency in NADPH oxidase

Answer 17

Chronic granulomatous disease

Question 18

Recurrent infection with Catalase positive organisms

Answer 18

Chronic granulomtous disease

Question 19

Absence of CD 18

Answer 19

Leukocyte adhesion defect

Question 20

Leukocytosis, failure to form pus, delay in umbilical cord sloughing

Answer 20

Leukocyte adhesion defect

Question 21

Granule structural defect

Answer 21

Chediak Higashi syndrome

Question 22

Recurrent bacterial infection , partial albinism

Answer 22

Chediak Higashi syndrome

Question 23

Deficiency in TNF gamna

Answer 23

Jobs syndrome

Question 24

Coarse facies, cold abscess, Inc IgE

Answer 24

Jobs syndrome