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Flashcards in Friday Review Deck (43):
1

In neutropenia, what are two ways that can cause decreased bone marrow production?

1. acquired
2. congenital

2

3 congenital disorders that can lead to neutropenia?

1. Kostmann syndrome
2. Shwachmann-Diamond
3. Cyclic neutropenia

3

Kostmann syndrome

- most severe congenital neutropenia
- dont make precursors
- G-CSF mutation

4

Shwachmann-Diamond syndrome

fat malabsoprtion
pancreatic insufficiency
bony abnormality
growth delay
autosomal recessive

5

Cyclic neutropenia

linked to apoptosis in marrow

6

In neutropenia, what are two ways that can cause increased turn over rate?

1. Immune
2. Non immune

7

3 examples of syndromes that cause autoimmune neutropenia (increased turnover rate)

SLE, Evan's syndrome, Felty's syndrome

8

What does G-CSF do?
How?

Treat neutropenia

§ Give G-CSF at 3-5 μg/kg daily or every other day (stimulating factor -tell BM to make more neutrophil)

9

What does "left shift" indicate?

increase in segs and bands (Neutrophils)

*think shifting left towards the start - the first responders

10

Basophilia cause by?

primarily food or drug hypersensitivity

11

Eosinophilia cause by?

allergic disorders, *parasitic* infections, and drug reactions

12

Monocytosis caused by?

• Hematologic disorders, lymphomas
• Collagen Vascular Disease
• Granulomatous Disease
• Infection
• Malignancy

13

Chediak Higashi syndrome
-what type of disorder is it?
-molecular defect
-functional defect
-clinical presentation
-does it cause neutropenia or neutrophilia?

Granule defect disorder

-molecular defect: alteration in membrane fusion -> giant leaky granules
-functional defect: neutropenia, large leaky granules that dont work
-clinical presentation: hepatosplenomegaly:
Oculocutaneous albinism, nystagmus photophobia. Recurrent infections of skin, ect.

causes neutropenia (only one of the neutrophil disorders that do)

14

Myeloperoxidase deficiency
-what type of disorder is it?
-molecular defect
-functional defect
-clinical presentation

-what is it? granule defect disorder


-functional defect: deficiency myeloperoxidase (killing bacteria power)

-clinical presentation: overall healthy, increased fungal infection, associated with diabetes

"myelo, my little toe has gangrene"

15

Leukocyte adhesion deficiency I
-what type of disorder is it?
-molecular defect
-functional defect
-clinical presentation

adherence disorder "the -itis"

-functional defect:
Neutrophilia.
Decreased adherence to endothelial surface leading to a defect in movement of neutrophils to infected tissue sites.
(especially bad after surgery)

-clinical presentation:
Recurrent soft tissue infections (skin, mucous membranes), gingivitis, mucositis, peridontitis, cellulitis, abscesses.
Delayed separation of umbilical cord/omphalitis.
Poor wound healing.

16

IFN gamma is made by which helper t cell

Th1

17

IL-2 is made by which helper t cell

Th1

18

2 ways that CTL can give "kissof death"

1. CTL's fas ligand (CD95L) binds "death receptor" Fas (CD95) on target→ linkage activates apoptosis pathway

2. Secrete lytic granules (containing granzyme proteases) → Secrete perforins to allow granzymes to penetrate → proteases trigger apoptosis

19

Useful markers on B cells:

CD20

20

Useful markers on T cells:

○ CD3 → found on all T cells
§ It's the transducer of the antigen-binding signal to inside the cell.
○ CD4 → found on all T helpers
○ CD8 → found on CTL

21

Chemokine function

cause inflammation
(small short mediators)

22

Cytokine function

affect behavior of same or another cell

23

Main lymphokines made by Th1, Th2, Th17 and Treg cells, and their biological functions.

1. Th1 → interferon gamma IFNγ and IL-2 →Pro-inflammatory→ Attracts/activates M1 macrophages

2. Th2 → Interleukin 4 (IL-4) →Pro-inflammatory →attracts/activates M2 macrophages

3. Th17 → Interleukin 17 (IL-17) → Attracts/activates M1 macrophages

4. Treg → TGFβ →FoxP3 → (IL-10) → Anti-inflammatory cytokine

24

2 types of mitogens
-what do they do?

2 plant lectins: PHA, PWM

-cause t cell mitosis:
Essentially they 'fool' every T cell they contact into thinking they're being bound by antigen.

25

Diff between mitogen and antigen?

mitogen doesnt actually bind to site on T cell (only binds CD3 domain that controls signal tranduction from antigen binding chains)

26

CTL recognize what class?
Helper T cells?

CTL: class I *think that there is only one type of killer T vs 5 helper*

Helper T cells: Class II

27

Which class of MHC is intrinsic pathway? Extrinsic?

Class I MHC "intrinsic pathway"
- Allows cells to say, "look what I've been making" (not endocytosed)
- All cells have Class I
- Class I is regulated by CTL
- CTL have CD8


Class II MHC "extrinsic pathway"
- "look what I've been eating"
- Professional APC (ie: dendritic cell)
- Helper T cells are activated by Class II

28

Which immunoglobins responds most to T independent antigens?

IgM
-makes sense bc IgM is the first Ig.

29

Which HLA make up class I MHC?

HLA-A

HLA-B

30

Which HLA make up class II MHC?

HLA D

31

Which class of MHC do APCs have?
Who recognizes it?

Class II MHC
-recognized by T-helper cells

32

Which types of cells have Class I MHC?
Who recognizes it?

all nucleated cells
-recognized by CTLs

33

If you are a helper cell, which part of you recognizes MHC type II?

CD4

34

HLA-D are all part of the Class __?__ MHCs

Class II MHCs

35

Which HLA is mainly involved in transplant?

HLA-DR

36

Which HLA is mainly involved in autoimmune diseases?

HLA-DP and DQ
*but not always..... or hardly ever on his table

37

Which HLA leads to type I diabetes?

HLA DQ

38

If Th1 is activated, and CTLs are not during a transplant, what happens?

Rej

39

Which is the most important to match for transplant? MHC class I or II?

It would be preferential to match MHC Class II (HLA-D match)

40

If HLA-D match but HLA-A and B do not:

* preferential
○ No MLR (mixed leukocyte rxn)
○ No Th1 activation and therefore no IL-2 produced
○ Few CTLs activated (bc needs IL-2)

MOST IMPORTANT TO MATCH for transplant

41

If HLA-A and B match but HLA-D do not:

○ Activate Th1 cells
○ No direct CTLs activated
○ Rejection still occurs, but more slowly

42

in T cell selection, how many points of recognition are there? Where?

6 CDRs total
-4 bind to MHC
- 2 bind to peptide

43

Which HLA is involved w/ Ankylosing spondylitis?

HLA-B27

-90% greater chance of getting AS if you have the gene