3 Immune Deficiencies

Question 1

What are some clinical features that are suggestive of immune deficiencies?

Answer 1

increased susceptibility to infection, autoimmune or inflammatory disorders, increased susceptibility to malignancies, syndrome complex

Question 2

what are some common characteristics of infection in immunodeficient states?

Answer 2

infections are recurrent, occur in multiple sites over time, caused by less virulent organisms, are unusually severe (or very occasionally less severe), and are difficult to treat

Question 3

What is the most common presenting manifestation of immunodeficient states?

Answer 3

infection

Question 4

name the typically sterile areas of the body

Answer 4

lower respiratory tract, GU tract, blood, CSF, joint fluid, pleural and peritoneal cavities

Question 5

in which areas of the body would you expect to find staphylococci and streptococci as normal flora?

Answer 5

skin and hair follicles

Question 6

H. influenzae, N. Meningitidis, and pneumococci are examples of normal flora in which area of the body?

Answer 6

oral cavity

Question 7

name an example of normal flora in the blood and CSF.

Answer 7

Trick question, there are none.

Question 8

T/F: antibiotics typically do not have an effect on normal flora in the body.

Answer 8

F–antibiotics can alter normal flora drastically.

Question 9

T/F: Immunosuppressed patients often develop infections at sites normally colonized by organisms

Answer 9

T

Question 10

Gram neg rods, anaerobes, and micro-aerophilic streptococci are normal flora in which area of the body?

Answer 10

GI tract

Question 11

what is the difference between primary and secondary immunodeficiency?

Answer 11

primary is inherited/genetic, and secondary is acquired (eg AIDS)

Question 12

what are the effector based classifications of immunodeficiency states?

Answer 12

antibody (B cell) disorders, cell mediated immunity (T cell) disorders, phagocytic disorders, complement disorders, and combined B and T cell disorders

Question 13

is prematurity an immunodeficient state?

Answer 13

yes, the more immature the newborn, the more immunocompromised

Question 14

All children have some form of immune system impairment until what age?

Answer 14

2 years old (due to lack of marginal b cells)

Question 15

what type of host defense mechanism defect results in multiple infections from indigenous flora at the site of impairment?

Answer 15

anatomic defects

Question 16

A defect in humoral immunity typically results in infection with what type of organism?

Answer 16

encapsulated bacteria

Question 17

what are the two types of humoral immunity defects?

Answer 17

antibody defects and complement defects

Question 18

infections in patients with antibody defects tend to occur in which areas of the body?

Answer 18

pulmonary tree, meninges, blood, GI

Question 19

infections in patients with cell mediated immunity defects tend to occur in which areas of the body?

Answer 19

lungs, meninges, GI, skin

Question 20

infection by parasites and protozoa is indicative of a defect in what type of immunity?

Answer 20

cell mediated immunity

Question 21

T/F: infection by extracellular bacteria is common in patients with cell mediated immunity deficiencies

Answer 21

F, intracellular bacterial infections are more common, along with fungi, DNA viruses, and some protozoa and parasites

Question 22

quantitative and qualitative PMN defects commonly lead to infections in which areas of the body?

Answer 22

blood, lungs, skin

Question 23

T/F: Both absolute number of PMNs and duration of neutropenia are important determinants of for risk and severity of infection.

Answer 23

T

Question 24

What are the two main organs of the reticulo-endothelial system (RES)?

Answer 24

spleen and liver

Question 25

T/F: defects in the reticuloendothelial system (RES) commonly lead to infections in the GI tract

Answer 25

F; RES defects lead to infections in the blood

Question 26

what organisms commonly cause infections in patients with defects in splenic immunity?

Answer 26

encapsulated bacteria (esp S pneumoniae

Question 27

what organisms commonly cause infections in patients with defects in hepatic immunity?

Answer 27

gram neg rods

Question 28

AIDS, treatment with immunosuppressive agents, and DiGeorge Syndrome all cause what type of immune system defect?

Answer 28

cell mediated immune defects

Question 29

Name some typical examples of quantitative and qualitative PMN defects.

Answer 29

Quantitative: consequence of chemotherapy
Qualitative: Chronic granulomatous disease (CHD), MPO deficiency, Hyper IgE syndrome, LAD

Question 30

Name some examples of anatomic problems that could cause recurrent infection

Answer 30

right middle lobe syndrome, recurrent cellulitis, kidney stones, retained foreign bodies

Question 31

what are some examples of splenic RES dysfunction?

Answer 31

congenital or surgical asplenia, hemoglobinopathies, sickle cell anemia

Question 32

T/F: Cirrhosis can cause hepatic RES dysfunction

Answer 32

T

Question 33

Bruton’s E-linked Agammaglobulinemia, X-linked Hyper IgM syndrome, and Selective IgA Deficiency are all examples of what type of immune deficiency?

Answer 33

Antibody deficiency

Question 34

Childhood AIDs, X-linked SCID, and MHC deficiency syndromes are all examples of what type of immune defect?

Answer 34

combined immunodeficiency syndromes