Immunodeficiency - lecture 1

Question 1

leucocyte adhesion deficiencies

Answer 1

disorders of phagocyte migration - failure to express adhesion molecules on vascular endothelium results in inability of phagocytes to exit blood stream.

Question 2

which primary phagocyte deficiency lacks pus formation and neutrophil infiltration

Answer 2

leucocyte adhesion deficiencies

Question 3

which primary phagocyte deficiency has high neutrophil count in the peripheral blood

Answer 3

leucocyte adhesion deficiencies

Question 4

chronic granulomatous disease

Answer 4

failure of oxidative killing

results in inability to clear organisms - excessive inflammation and granulomatous formation

Question 5

which primary phagocyte deficiency shows abnormal results in NBT test

Answer 5

chronic granulomatous disease

Question 6

defects in cytokines and cytokines receptors

Answer 6

e.g. IFN - gamma and IL-12 or their receptors results in failure of intracellular killing

Question 7

which primary phagocyte deficiency makes individuals susceptible to mycobacterial infections

Answer 7

defects in cytokines and cytokines receptors

Question 8

3 types of primary phagocyte deficiency

Answer 8

chronic granulomatous disease
leucocyte adhesion deficiency
defects in cytokines and cytokines receptors

Question 9

complement pathway deficiency causes individuals to present with recurrent infection with ________ bacteria

Answer 9

encapsulated

Question 10

deficiency of classical complement pathway (C1, C4, C2) is associated with

Answer 10

autoimmune disease

Question 11

individuals with mannose binding lectin deficiency have increase incidence of _____ infection if subjected to _______

Answer 11

bacterial

additional cause of immunocompromised e.g. premature birth or chemotherapy

Question 12

primary deficiencies of adaptive immune system - 3 examples

Answer 12

digeorge syndrome
bare lymphocyte syndrome
autoimmune lymphoproliferative syndrome

Question 13

digeorge syndrome caused by

Answer 13

failure of development of pharyngeal pouch, usually cause by deletion of 22q11

Question 14

digeorge syndrome characterised by

Answer 14

low numbers of mature T cells

Question 15

bare lymphocyte syndrome characterised by

Answer 15

absent expression of HLA molecules in thymus

Question 16

bare lymphocyte syndrome - failure of HLA class I to develop causes

Answer 16

failure of CD8+ T cells to develop

Question 17

bare lymphocyte syndrome - failure of HLA class II to develop causes

Answer 17

failure of CD4+ T cells to develop

Question 18

autoimmune lymphoproliferative syndrome is

Answer 18

failure of apoptosis of lymphocytes

Question 19

combined T and B lymphocyte deficiencies

Answer 19

severe combined immune deficiency (SCID) is caused by failure of T and B cells to mature

Question 20

treatment for SCID

Answer 20

bone marrow transplant

Question 21

primary antibody deficiencies - 3 examples

Answer 21

selective IgA deficiency
common variable immune deficiency
specific antibody deficiency / functional IgG deficiency

Question 22

most common primary antibody deficiency

Answer 22

selective IgA deficiency

Question 23

selective IgA deficiency - sometimes compensatory increase in ___ levels

Answer 23

IgG levels

Question 24

CVID characterised by

Answer 24

low serum IgG levels

failure to make antibody response to exogenous pathogens

Question 25

specific antibody deficiency / functional IgG deficiency characterised by

Answer 25

defective antibody responses to polysaccharide antigens

Question 26

secondary immune deficiency cause by

Answer 26

external factors

Question 27

common physiological causes of secondary immune deficiency

Answer 27

age
premature birth
pregnancy

Question 28

common infection causes of secondary immune deficiency

Answer 28

HIV
measles
mycobacterial infection

Question 29

common iatrogenic causes of secondary immune deficiency

Answer 29

IS therapy 
corticosteroids 
stem cell transplantation 
radiation injury 
anti-epileptic agents

Question 30

common malignancy causes of secondary immune deficiency

Answer 30

B cell malignancies - leukaemia, lymphoma, myeloma (bone marrow cancer)
solid tumours