FA quick B cell disorders 03-29 (2)

Question 1

3 B cell disorders?

Answer 1

X-linked (Bruton) agammaglobulinemia;
Selective IgA deficiency;
Common variable immunodeficiency

Question 2

Defect in BTK, a tyrosine kinase gene?

Answer 2

X-linked (Bruton) agammaglobulinemia

Question 3

Defect in BTK, a tyrosine kinase gene –> no B-cell maturation?

Answer 3

X-linked (Bruton) agammaglobulinemia

Question 4

No B cell maturation?

Answer 4

X-linked (Bruton) agammaglobulinemia

Question 5

X-linked recessive ( inc. in Boys)?

Answer 5

X-linked (Bruton) agammaglobulinemia

Question 6

Recurrent bacterial and enteroviral infections after 6 months (decr. maternal IgG)

Answer 6

X-linked (Bruton) agammaglobulinemia

Question 7

Absent B cells in peripheral blood, decr. Ig of all classes?

Answer 7

X-linked (Bruton) agammaglobulinemia

Question 8

Absent/scanty lymph nodes
and tonsils (1° follicles and germinal centers absent) --> live vaccines contraindicated?

Answer 8

X-linked (Bruton) agammaglobulinemia

Question 9

live vaccines contraindicated?

Answer 9

X-linked (Bruton) agammaglobulinemia

Question 10

Most common 1° immunodeficiency?

Answer 10

Selective IgA deficiency

Question 11

Majority Asymptomatic?

Answer 11

Selective IgA deficiency

Question 12

Can see Airway and GI

infections, Autoimmune disease, Atopy, Anaphylaxis to IgA in blood products?

Answer 12

Selective IgA deficiency

Question 13

Anaphylaxis to IgA in blood products?

Answer 13

Selective IgA deficiency

Question 14

decr. IgA with normal IgG, IgM levels

Answer 14

Selective IgA deficiency

Question 15

incr. susceptibility to giardiasis

Answer 15

Selective IgA deficiency

Question 16

Can cause false-negative celiac disease test?

wtf celiac, siaip nestumo testa

Answer 16

Selective IgA deficiency

Question 17

Defect in B-cell differentiation.

Answer 17

Common variable immunodeficiency

Question 18

May present in childhood but usually diagnosed after puberty?

Answer 18

Common variable immunodeficiency

Question 19

incr. risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections

Answer 19

Common variable immunodeficiency

Question 20

decr. plasma cells, decr. immunoglobulins

Answer 20

Common variable immunodeficiency

Question 21

incr. risk of autoimmune disease?

Answer 21

Common variable immunodeficiency

Question 22

incr. risk of bronchiectasis?

Answer 22

Common variable immunodeficiency

Question 23

incr. risk of lymphoma?

Answer 23

Common variable immunodeficiency

Question 24

incr. risk of sinopulmonary infections?

Answer 24

Common variable immunodeficiency

Question 25

Can see Airway and GI

infections?

Answer 25

Selective IgA deficiency

Question 26

Can see Autoimmune disease?

Answer 26

Selective IgA deficiency

Question 27

Can see Atopy?

Answer 27

Selective IgA deficiency

Question 28

Can see Anaphylaxis to IgA in blood products?

Answer 28

Selective IgA deficiency