Immune Deficiency

Question 1

B cell surface proteins

Answer 1

CD19 or CD20

Question 2

helper T cell surface proteins

Answer 2

CD3 and CD4

Question 3

cytotoxic T cell surface proteins

Answer 3

CD3 and CD8

Question 4

NK cell surface proteins

Answer 4

CD15 and CD56

Question 5

IgG

Answer 5

most common
monomer
phagocytosis - opsonisation and neutralisation

Question 6

IgA

Answer 6

mucous membranes

monomer/dimer

Question 7

IgM

Answer 7

precursor of IgG
1st response to antigens
pentamer

Question 8

IgE

Answer 8

response to allergens

monomer

Question 9

IgD

Answer 9

on the plasma membranes of immature B lymphocytes

monomer

Question 10

primary immune deficiencies

Answer 10

Ab def.
T cell immune def.
Complement def.
Phagocyte def.

Question 11

secondary immune deficiencies

Answer 11

Infection
lymphoproliferative disease
HIV
immunosuppresant drugs
protein losing states
leukaemia
lymphoma
catabolic/nutricional deficiencies

Question 12

SIgAD

Answer 12

Specific IgA Deficiency
primary humoural def.
common
IgA in resp and GI mucosal surfaces therefore more prone to infections there
usually needs no treatment

Question 13

XLA

Answer 13

X-linked agammaglobulinaemia
primary humoural def.
mutation in BTK gene
pro-B –X–> mature B cell, therefore no peripheral B cells in blood, therefore no Ig production

Question 14

CVID

Answer 14

Common variable immune deficiency
primary homoural def.
reduction in either IgG, IgA and or IgM
umbrella term where no other primary or secondary cause identified

Question 15

Transient hypogammaglobulinaemia of childhood

Answer 15

period of low IgG in early childhood
primary humoural def.
when maternal IgG levels start to drop and the child’s own aren’t high enough

Question 16

examples of lymphoproliferative disease (secondary humoural def)

Answer 16

Chronic lymphocytic leukemia (CLL)

multiple myeloma

Question 17

causes of iatrogenic secondary immune def.

Answer 17

immunosupressing drugs

Question 18

causes of protein loss secondary immune def.

Answer 18

burns, renal failure, GI loss

remember Ab are proteins!

Question 19

Treatment of humoural (Ab) deficiency

Answer 19

Ig Replacement therapy from donor blood, IgG only
Chest management
Prophylactic antibiotics
SIgAD often needs no treatment

Question 20

causes of complement def.

Answer 20

secondary - active infection/inflammation causes them to be used up, therefore causeing a def.

Question 21

what do complements do?

Answer 21

complement system important in promoting inflammation, activating phagocytes, killing pathogens, assisting with foreign/damaged tissue

Question 22

C3 deficiency can occur secondary to…

Answer 22

streptococcal glomerulonephritis

Question 23

C3/4 deficiency can occur secondary to…

Answer 23

lupus (SLE) nephritis

Question 24

C2-4 deficiency can cause…

Answer 24

susceptibility to bacterial infection and autoimmmune disease (lupus)

Question 25

C5-9 deficiency can cause…

Answer 25

recurrent meningococcal disease

Question 26

C1 INHIBITOR deficiency can cause… and is treated with…

Answer 26

hereditary/aquired angioedema
oedema of face/extremities

treated with steroids and transexamic acid.
rescue treatment of C1 inhibitor concentrate

Question 27

phagocytes - disorders of neutrophils

Answer 27

reduction in number - congential/chemotherapy
abnormal function - chronic granulomatous disease ( consider BMT/gene therapy)
leads to an increase in susceptibility to pyogenic bacteria and intracellular organisms

Question 28

SCID

Answer 28

severe combined immunodeficiency
cell mediated immune response (T cell deficiency)
infancy, failure to thrive
lymphopenia +/- hypogammaglobulinaemia - absent or non functioning T cells (sometimes with absent B or NK cells)
managed with isolation, antimicrobial therapy, Ig replacement, BMT/stem cell therapy