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Flashcards in Lecture 19 Thrush Deck (21):
1

Primary Immunodeficieny

inherited/ due to genetics

2

Secondary immunodeficiency

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

3

Fatal defect that affects both B and T cells

defect without RAG

4

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

unable to produce immunoglobins, IgA most common
When all are not produced= patients have bacterial infections especially the encapulated bacteria

5

T cell def.

worst
result in intracellular infections
decrease in overall Ig production

6

Even if T cells defective - newborn

mother's antibodies can help child

7

Myeloid deficiencies

problem with phagocytosis
increase in bacterial infections

8

SCID

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

9

Types of SCID

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

10

Autosomal SCID

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

11

WAS Wilc aldrich

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

12

DiGeorge

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

13

Ataxia

breakage at site of TCR
no t and b cells

14

Bare lymphocyte syndrome

defec in MHC II

15

TAP

lack of MHC I

16

Bruton's hypogammaglobulinemia

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

17

X linked hyper IgM immunodef.

high IgM and low IgA/ IgG (no swtching)
defect in CD40L on t cell

18

IgA deficiency

most common
can be asymptomatic
resp. UTI problems

19

Myeloid (Chronic Granul. Disease)

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

20

Example of Secondary Immunodef.

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

21

Therapy for Immunodef.

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