Friday Review Flashcards
In neutropenia, what are two ways that can cause decreased bone marrow production?
- acquired
2. congenital
3 congenital disorders that can lead to neutropenia?
- Kostmann syndrome
- Shwachmann-Diamond
- Cyclic neutropenia
Kostmann syndrome
- most severe congenital neutropenia
- dont make precursors
- G-CSF mutation
Shwachmann-Diamond syndrome
fat malabsoprtion pancreatic insufficiency bony abnormality growth delay autosomal recessive
Cyclic neutropenia
linked to apoptosis in marrow
In neutropenia, what are two ways that can cause increased turn over rate?
- Immune
2. Non immune
3 examples of syndromes that cause autoimmune neutropenia (increased turnover rate)
SLE, Evan’s syndrome, Felty’s syndrome
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)
What does “left shift” indicate?
increase in segs and bands (Neutrophils)
*think shifting left towards the start - the first responders
Basophilia cause by?
primarily food or drug hypersensitivity
Eosinophilia cause by?
allergic disorders, parasitic infections, and drug reactions
Monocytosis caused by?
- Hematologic disorders, lymphomas
- Collagen Vascular Disease
- Granulomatous Disease
- Infection
- Malignancy
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)
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”
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.
IFN gamma is made by which helper t cell
Th1
IL-2 is made by which helper t cell
Th1
2 ways that CTL can give “kissof death”
- CTL’s fas ligand (CD95L) binds “death receptor” Fas (CD95) on target→ linkage activates apoptosis pathway
- Secrete lytic granules (containing granzyme proteases) → Secrete perforins to allow granzymes to penetrate → proteases trigger apoptosis
Useful markers on B cells:
CD20
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
Chemokine function
cause inflammation (small short mediators)
Cytokine function
affect behavior of same or another cell
Main lymphokines made by Th1, Th2, Th17 and Treg cells, and their biological functions.
- Th1 → interferon gamma IFNγ and IL-2 →Pro-inflammatory→ Attracts/activates M1 macrophages
- Th2 → Interleukin 4 (IL-4) →Pro-inflammatory →attracts/activates M2 macrophages
- Th17 → Interleukin 17 (IL-17) → Attracts/activates M1 macrophages
- Treg → TGFβ →FoxP3 → (IL-10) → Anti-inflammatory cytokine
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.
