Lecture 19 Thrush

Question 1

Primary Immunodeficieny

Answer 1

inherited/ due to genetics

Question 2

Secondary immunodeficiency

Answer 2

aquired
due to infections
due to aging
due to malignency
associated with cancer

Question 3

Fatal defect that affects both B and T cells

Answer 3

defect without RAG

Question 4

What do B cell deficiencies cause? What is the most common?

Answer 4

unable to produce immunoglobins, IgA most common

When all are not produced= patients have bacterial infections especially the encapulated bacteria

Question 5

T cell def.

Answer 5

worst
result in intracellular infections
decrease in overall Ig production

Question 6

Even if T cells defective - newborn

Answer 6

mother’s antibodies can help child

Question 7

Myeloid deficiencies

Answer 7

problem with phagocytosis

increase in bacterial infections

Question 8

SCID

Answer 8

defect in T cells (may or may not affect B cell and NK ceels)
not affect myeloid
low number of lymphocytes
infecition usuallly viral or fungal not bacterial (due to mom passive IgG )
can treat with bone marrow transplant

Question 9

Types of SCID

Answer 9

Jak/ x linked gamma
RAG1/ RAG2 (both t and b)
ADA
CD3 
CD 45
IL 7

Question 10

Autosomal SCID

Answer 10

RAG1/ RAG2- affects both B and T
ADA: affects CD25- DNA synthesis/ other cells
alpha IL 2- CD25 (t cells and t regs)

Question 11

WAS Wilc aldrich

Answer 11

• affects actin
• more severe with age
• x linked
• loss of t and b funcion
• platelet def.

Question 12

DiGeorge

Answer 12

• loss of alpha beta t cells
• t cells only
• lack of thymus
  treated by thymic graft

Question 13

Ataxia

Answer 13

breakage at site of TCR

no t and b cells

Question 14

Bare lymphocyte syndrome

Answer 14

defec in MHC II

Question 15

TAP

Answer 15

lack of MHC I

Question 16

Bruton’s hypogammaglobulinemia

Answer 16

x linked
lack of antibodies
b cell defect
no peripheral b cells
btk gene 
h chain
recurrent bacterial infections

Question 17

X linked hyper IgM immunodef.

Answer 17

high IgM and low IgA/ IgG (no swtching)

defect in CD40L on t cell

Question 18

IgA deficiency

Answer 18

most common
can be asymptomatic
resp. UTI problems

Question 19

Myeloid (Chronic Granul. Disease)

Answer 19

phagocytic cells (neutrophils) are not able to produce hydrogen peroxide/reactive oxygen products due to a defect in NADPH oxidase activity. This is confirmed by a negative result in the nitroblue tetrazolium dye (NBT) reductase test. If an individual makes the ROS, then the dye will be reduced and turn blue.

IFNγ treatment has been shown to stimulate normal production of superoxide in some of these patients

Question 20

Example of Secondary Immunodef.

Answer 20

HIV
decrease in CD4 t cells
causes opportunistic inhections
under 400/ under 200

Question 21

Therapy for Immunodef.

Answer 21

genetic engineering
bone marrow transplant (wont work for digeorge)
Passive: IV, cytokines etc