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I3 > Week 1 > Flashcards

Week 1 Flashcards

1
Q

adult blood volumnes

A

male = 5-6L, female = 4-5L

– be careful taking blood from newborns. duh.

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2
Q

hematocrit

A

% of blood volume that is RBCs

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3
Q

plasma vs serum

A

serum = plasma without clotting factors.

get serum by letting blood stand and clot, and spinning it down. serum used for most metabolic panels

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4
Q

blue-top tube

A

blood anti-coagulated with Na-Citrate.

used to measure functionality of coagulation cascade in plasma

PT (prothrombin time)
PPT (partial thromboplastin time)

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5
Q

purple-top tube

A

blood anti-coagulated with Na-EDTA

used to assess cells in the blood - RBCs, WBCs, platelets

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6
Q

Problems with CBC

A

Costs a lot, not always recommended. not recommended for healthy or mildly ill patients - will find things abnormal by chance - need to know what you’re looking for before ordering one.

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7
Q

when to order CBC

A

for patients for which you suspect anemia (low RBC), thrombocytopenia (low platelet), leukopenia (low WBC), serious infection (not specific) or systemic inflammatory disease

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8
Q

differential tree for anemia

A
  1. high/low reticulocyte (bone marrow production)

2. if low, MCV, if high, look for bleeding or hemolysis

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9
Q

WBC components (percentages) in peripheral blood

A 
neutrophils (40-75)
eosinophils (1-6)
basophil (<1)
monocytes (2-10)
lymphocytes (20-50- B and T cells)
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10
Q

what cells come from myeloid progenitor

A

erythrocytes, platelets (from megakaryocyte), macrophages (from monocytes), neutrophils, mast cells, and eosinophils

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11
Q

which cells come from lymphoid progenitor

A

plasma cell (activated B cell), T cell, NK cells

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12
Q

where do certain WBCs differentiate/ become activated

A

B/T/NK get activated in lymphoid tissue and then migrate to peripheral tissues/inflammatory sites

Macrophages get activated in a tissue specific fashion

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13
Q

macrophage morphology and function

A

look different in different tissues.

hallmark features: big cells, lots of cytoplasm

function: phagocytose particles, make cytokines, present antigens to immune cells

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14
Q

dendritic cell morphology and function

A

related to macrophages, present in tissues.

interact with immune cells, make cytokines

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15
Q

neutrophil morphology and function

A

moslty in blood

have 3 kinds of granules
last 1-4 days

polynucleated lobes, purplish granules

can migrate to tissue and phagocytose

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16
Q

eosinophil morphology and function

A

look like neutrophil, but only have two lobes, and have denser coarser granules - pink staining.

circulate in blood, migrate to tissue, excrete granular content to mediate inflammatory response

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17
Q

basophils morphology and function

A

very rare. can’t see nucleus because of basophilic granules.

circulate in blood. mediate allergic inflammatory responses. granules similar to mast cells

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18
Q

mast cell morphology and function

A

basophilic granuels , reside in tissue.

mediate allergic response

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19
Q

lymphocyte morphology and function

A

small, have little cytoplasm and large nucleus. RBC diameter similar to nucleus in lymphocyte

immune response

NK and cytotoxic T cells have more cytoplasm and have small granules

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20
Q

monocyte morphology and function

A

kidney bean shaped nucleus, large, in blood.

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21
Q

CD nomenclature

A

“cluster of differentiation”

number proteins on cells - protein expression varies depending on cell and stage of differentiation

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22
Q

What protein is expressed on all mature T cells

A

CD3

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23
Q

what proteins are expressed by helper T cells

A

CD3 and CD4

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24
Q

what proteins are expressed by cytotoxic T cells

A

CD3 and CD8

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25
Q

what proteins are expressed by all B cells

A

CD19 (are CD3 negative)

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26
Q

what proteins are expressed by all NK cells

A

CD16/56 (are CD3 negative)

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27
Q

blood constitutes what percentage of total body weight

A

7-8%

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28
Q

examples of molecules in colloidal disperion

A

albumin, fibrinogen, globulin

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29
Q

size and notable features of RBCs

A

7.5 micrometers +/- 1.5 micrometer

takes of giemsa

average lifespan is 100-120 days, high turnover

have tubules, lose mitochondria, have central hemoglobin (33% solution- almost saturated)

use glucose, contain vitamins and bicarb

plasma membrane of RBCs have mostly lipid, then protein, then carbs. the ABO blood groups are 8% of the glycocalyx on surface

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30
Q

function of RBC

A

3 Cs
carry respiratory gasses
carry antigenic determinants
contain hemoglobin as buffer

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31
Q

changes in RBC count

A

increases (leukocytosis) NORMALLY during exercise, high altitude, pregnancy, dehydration and in neonates

increases ABNORMALLY during heart failure, hypoxia, and polycythemia vera

decreases (leukopenia) are ALWAYS PATHOLOGICAL - due to anemia (hemorrhage, destruction of bone marrow, iron deficiency)

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32
Q

change in RBC morphology

A

sickle cell

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33
Q

granular vs agranular leukocytes

A

granular (AKA polymorphonuclear PMN cells) (neutrophils, eosinophils, basophils)

agruanular (lymphocytes, monocytes - have lysosomes)

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34
Q

normal hematocrit

A

40-45% (RBC percentage)

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35
Q

different parts of platelets

A

hyalomere and granulomere

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36
Q

von willebrand factor

A

contained in membrane bound bodies in endothelium .

clotting factor in large vessels

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37
Q

Functions of endothelium - 7 functions

A
  1. selective permeability (diffusion, active transport, pinocytosis, coated pits, fenestration)
  2. thrombosis control (anti-thrombogenics and coagulants)
  3. pro-thrombogenics - clotting
  4. BP regulation - vasoconstrictors, vaso dilators
  5. modulation of immune and inflammatory response - leukocyte adhesion, interleukins for migration
  6. hormonal regulatory factors (GFs, IFs, ACE - angiotensin 1->2, inactivators)
  7. lipoprotein oxidation (LDLs, VLDLs, cholesterol are oxidized by free radicals and make foam cells - plaque formation)
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38
Q

layers of vessels

A
  1. tunica intima (endothelium)
  2. tunica media (concentric smooth muscle - more in arteries)
  3. tunica adventitia (longitudinal CT - more in veins)
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39
Q

properties of lymphatic vessels

A
  • used for big particles (both good and bad)
  • able to distinguish good and bad
  • large openings, irregular outlines, with anchoring fibers
  • large vessels have valves
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40
Q

connection between lymph and circulatory system

A

thoracic duct, right lymphatic duct - lymph collects and drains into venous system (subclavian vein)

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41
Q

spleen functions

A
  1. hemopoietic - destruction of RBCs and retrieval of iron from hemoglobin
  2. immune function
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42
Q

spleen histology (pulp)

A
  1. red pulp - RBC (vascularized pulp peripheral to PALS)
  2. white pulp - immune (contains T and B cells, responds to antigens, forms PALS around arteries - activated has lighter crown and darker mantle)
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43
Q

capsule and trabeculae

A

trabeculae is capsule invaginations - inner structure

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44
Q

rapid and slow route in splenic capillary beds - red pulp

A
  1. rapid route - not found in humans – closed circulation - straight through
  2. slow route - sinusoids with fenestrations, into cords of bilroth (you get picked up back into sinus if you’re a healthy RBC, if you’re not, you don’t go back into circulation)
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45
Q

3 functions of hemoglobin

A
  1. oxygen binds to heme (Fe2+)
  2. CO2 binds N-terminal amine group of 4 globin subunits forming carbamino group
  3. hemoglobin acts as pH buffer by transporting H+ via histidine
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46
Q

exchange of gasses via hemoglobin - step by step (5 steps)

A
  1. in lungs, pO2 is high, so O2 passes into RBC, binding to deoxy-Hb, making oxy-Hb
  2. oxy-Hb is a stronger acid than deoxy-Hb and it gives up H+, shifting equilibrium between bicar and carbonic acid toward carbonic acid
  3. carbonic acid dissociates into CO2 and water - catalyzed by carbonic anhydrase
  4. low pCO2 drives CO2 out of RBC and into lung
  5. bicarb/chloride antiport transports bicarb out and chloride in (maintain ionic balance and make sure no lysis happens - chloride shift)
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47
Q

3 basic classification of a anemias

A
  1. normocytic normochromic anemia - normal size normal appearance, low hematocrit (<40%) can come from hemolytic anemia (can occur due to loss of pyruvate kinase) and blood loss.
  2. microcytic hypochromic anemia - small size and under colored - low to normal hematocrit, less hemoglobin or heme synthesis occuring
  3. macrocytic normochromic anemia (megaloblastic) - inhibited cell division - seen because of folate/vit B12 deficiency
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48
Q

from pleuripotent stem cell to RBC

A
  1. pleuripotent stem cell
  2. unipotent stem cell
  3. proerythroblast (large neucleus)
  4. early normoblast (basophilic, start heme synthesis)
  5. intermediate normoblast - getting smaller
  6. last normoblast (orthochromic) - even smaller - nucleus get extruded
  7. reticulocyte (no nucleus, but ribosomes form heme synthesis)
  8. erythrocyte RBC

takes several days - if this is blocked, by a week you’ll have lost 0.8 x 7 = 5.5% loss

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49
Q

B12 and folate and anemia

A

megaloblatic/
pernicious anemia: loss of gastric parietal cells (bariatric sx, cancer, autoimmune) or deficiency of IF – causes megaloblastic anemia (don’t have enough folate/B12 to divide, so get large but can’t divide)

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50
Q

iron deficiency and anemia

A

microcytic hypochromatic anemia (pinker, smaller)

used in hemoglobin (65% of total body iron is in hemoglobin)

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51
Q

lead and heme synthesis

A

glycine + succinyl-coA –> 5-ALA (Rate limiting). enzyme for this step is 5-ALA synthase which is sensitive to lead.

ferrochelatase, which inserts Fe2+ into heme structure is also sensitive to lead.

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52
Q

porphobilinogen

A

build up causes pain and neurological issues. 2 ALA come together via 5-ALA dehydratase to form porphobilinogen

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53
Q

porphyria

A

caused by defective enzymes in heme biosynthesis - different kinds dependent on enzymes

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54
Q

deficiency in UP3 decarboxylase causes

A

cutanea tarda (Can be acquired - don’t need family history)

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55
Q

acute porphyria

A

includes acute intermittent, variegate, and herediatry corproporphyria

is episodic

see:
abdominal pian, purple splotches, red urine, muscle weakeness, psychotic episodes, anxiety, schizophrenia - king george 3

can prevent acute attack - by hemin

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56
Q

chronic porphyria

A

includes congenital erythropoietic porphyria, porhyria cutanea tarda, veriegate porphyria

long term stable

dermatological issues (light sensitivity, blistering, sloughing) - werewolf syndrome, body hair, vampire syndrome, red gums, stained teeth, light sensitivity

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57
Q

globin genes and order of expression

A

alpha gene found on chromosome 16, beta on chromosome 11
From left to right:
(chrom 16) zeta (fetal), alpha 2, alpha 1
(chrom 11) epsilon (fetal), gamma G, gamma A, delta, beta

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58
Q

mutations in alpha or beta globin is more dangerous

A

alpha, because beta has a lot more regulation and complexity and options (locus of control region for beta is extensive)

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59
Q

what causes beta thalassemia

A

point mutation in globin gene intron yielding alternate splice site - adds 21 nucleotides = in frame 7 AA shift

yeilds hemeoglobin that acts more like myoglobin and doesn’t like to give up oxygen

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60
Q

developmental heme switching

A

in fetus, epsilon and zeta (from yolk sac) are first to decrease, as alpha and gamma rise in the liver. beta starts to rise slowly and at around 7 weeks, the gamma/beta ratio is about half/half. bone marrow is taking over. delta rises slowly.

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61
Q

HbA1 (AKA HbA) contains which types of globin

A

2alpha2beta

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62
Q

HbA2 contains which types of globin

A

2alpha2delta

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63
Q

HbF contains which types of globin

A

2alpha2gamma

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64
Q

heme binding pocket contains

A

hydrophobic cleft and two central histidines:

proximal histidine binds covalently to Fe2+ and other protects the oxygen binding site (distal)

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65
Q

pO2 in muscle vs lung

A

muscle - 26-30torr
lung - 100-110torr

hemoglobin will give up oxygen that it gets in the lung in the muscle.

in muscle, hemoglobin is more stable as deoxygenated than oxygenated. myoglobin is more stable in oxygenated

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66
Q

cooperative interactions

A

subunit-subunit interactions
in deoxy, once the 1st O2 binds, the rest bind (distal histidine gets out of the way because of the binding of the first O2 and the conformational change)
in oxy, once the 1st O2 leaves, the rest leave (distal sweeps the O2s out of the way)

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67
Q

how many salt bridges in deoxy-form (T) of hemoglobin

A

8

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68
Q

how many salt bridges in oxy-form (R) of hemoglobin

A

6

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69
Q

heme metabolism and carbon monoxide

A
  1. hemoglobin -> globin and hemin
  2. hemin -> biliverdin and CO (from non-polar side of hemin) (via heme oxygenase)
  3. biliverdin -> bilirubin (via bilverdin reductase)

bilirubin has free rotation - clamps down on itslef - not soluble.

  1. in liver, bilirubin is modified by adding 2 glucuronic acids to make it soluble so it can be lot in the bile
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70
Q

How can Hb be forced to deliver more O2 to metabolically active peripheral tissues

A

increase the number of deoxy stabilizing salt bridges

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71
Q

bohr effect

A

increasing pCO2 and number of protons drives oxygen binding curve

H+ adds to histidine, establishing new salt bridge on each Beta – now at 10 salt bridges

CO2 binds amino terminal end of beta-globin making carbamate on each subunit making 4 additional salt links – now at 14 salt bridges

so, in acidic environment (lactic acidosis), you give off a lot of O2 and pick up CO2

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72
Q

chloride shift

A

as CO2 increases, HCO3- will flow out of cell into circulation, when that negative charge leaves, you create electrical gradient, so Cl- comes in. Cl- level in circulatory system drops.
H+ binds to heme stabilizing deoxy confirmation to give off O2

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73
Q

Rapoport-Leubering Shunt

A

in H+ conditions, H+ activates mutase which takes 1,3 BPG to 2,3 BPG and then back to 3-PGA by phosphatase

2,3 BPG gets into central pore of hemoglobin, stabilizing the deoxy conformation by adding 6 additional salt-bridges between beta-subunits – now have 18-20 salt bridges stabilizing the deoxy unit - forces all O2 off the hemoglobin

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74
Q

3 metabolic pathways in RBC

A
  1. glycolysis
  2. HMP shunt
  3. rapoport-leubering shunt
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75
Q

difference between fetal hemoglobin HbF and maternal HbA

A

fetal hemoglobin misses a histidine, so you have 2 fewer salt bridges - so deoxy form in mom is more stable - mom gives off O2 to fetus.

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76
Q

if you have a problem with Beta subunit, how does your body react

A

can induce expression of gamma and delta

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77
Q

sickle cell

A

position 6 on Beta chain, point mutation takes glutamic acid off the surface of heme, which was making it soluble - shell of hydration. without it, the heme sticks together. in deoxy states, heme can precipitate out of solution.

so for people with trait, don’t exert, don’t go to high altitudes

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78
Q

HbC

A

lysine replaces glutamic acid instead of valine.
lysine is positive - still has shell of hydration. for heterozygote, one is normal one is lysine - you get electrostatic interaction - make salt bridges, make heme to clump up - doesn’t spindle.

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79
Q

MetHb

A

1-2% normally is MetHb. Hb-O2 gets converted to Met-Hb and releases O2 radical. SOD takes care of radical, but at high levels can cause damage.
Met Hb doesn’t bind O2 - changes blood to blue-chocolate blood. Met Hb reductase uses cytochrome (uses NADH from glycolysis).
in cyanide poisioning, give amyl nitrite you get Met-Hb and then remove by nitrite and thiosulfate…. or just use B12

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80
Q

issue with RBC lysis

A

RBCs lyse and release hemoglobin, hemoglobin is right at the threshold for kidney filtration and ends up clogging it leading to kidney failure.

Normally, to prevent this, hemoglobin gets complexed with HAPTOGLOBIN which is synthesized by the liver, and the complex is large enough that it doesn’t damage the kidney, and gets targeted to spleen/liver for metabolism.

when lysis happens free heme is also picked up by HEMOPEXIN, to prevent clearance from kidney and to recycle iron. hemes create free radicals.

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81
Q

Hemoglobin is broken down into ___

A
  1. iron
  2. bilirubin (heme)
  3. globin (re-enters amino acid pool)
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82
Q

jaundice

A

AKA ICTERUS
excessive RBC turnover, hemolytic anemias, bile blockage, liver disease (blocked glucuronylation) all lead to buildup of insoluble bilirubin - accumulate in whites of eyes and fatty tissues under skin – leading to yellow coloration.

biliverdin absorbs light - neonatal jaundice - light therapy prevents conversion of biliverdin to bilirubin which is insoluble (because babies don’t have glucuronyosyltransferase). if not resolved, baby will have neurologic issues called KERNICTERUS

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83
Q

epo

A

erythrocytic growth factor - negative feedback like all the growth factors

mostly made in kidney, but can also be made by liver

epo producing cells have O2 sensors (not enough stimulates RBC production) - then downregulates neg feedback

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84
Q

bone marrow -

A

scaffolding for differentiation and regulation by cell-cell interactions - niche theory

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85
Q

niche theory

A

vascular niche
osteoblast niche

regulated by cytokines - tells cells to get out of niche and differentiate

provide backup supply

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86
Q

erythocytic lineage time to mature, #progeny, survival time

A

5 days
16 (4 divisions)
120 days

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87
Q

granulocytic lineage time to mature, #progeny, survival time

A

5 days (14 days to release)
16-32 (4-5 divisions)
1-4 days

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88
Q

megakaryocytic lineage time to mature, #progeny, survival time

A

5 days
hundreds (many fragmentations into platelets)
8-11 days ~ 10 days

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89
Q

which cells (what stage) can undergo mitosis

A

only ‘blasts and pro’ cytes

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90
Q

sites of hematopoesis from fetal to adult

A

yolk sac, then liver and spleen, bone marrow takes over by the time of birth.
adult (vertebral and pelvis (posterior iliac spine) first then sternum (can only do aspirate), then ribs) lymph nodes also.

long bones only active until puberty (anterior tibia)

less reserves in central bone as we age

spleen and liver have capacity to resume hematopoesis

91
Q

what can you see/assess with aspirate smear

A
  • ratio of M:E (normal is 3:1) granulocytes:erythocytes
  • maturation of lineages
  • low frequency elements (plasma, lymphocyte etc.)
  • iron stores (not decalcified like for core)
  • foreign cells (metastatic neoplasms)
  • microorganisms
92
Q

what stain do you use to see iron in aspirate smear

A

prussian blue

93
Q

what do we look for/see in bone marrow core

A
  • cellularity (~90% occupied by cells in children, ~30% in elderly - more fat in elderly)
  • maturation of lineages (aspirate is better)
  • fibrosis (only in core)
  • foreign cells (better than aspirate)
  • granulomas (only in core)
  • microorganisms
  • bone morphology
94
Q

when do you do a bone marrow examination

A

not first - it’s invasive
if you can’t explain it with other non invasive tests
1. combination of anemia, neutropenia and/or thrombocytopenia
2. leukemia eval
3. metastatic neoplasms (staging)
4. search for infectious agents (mycobacteria or fungi)

95
Q

definition of anemia

A

reduction of RBC mass, results in decreased ability to carry O2

96
Q

variables that affect hematocrit, hemoglobin and RBC count

A
  • gender (higher in men)
  • age
  • altitude (increased at high altitude)
  • posture (prolonged bed rest decreases)
  • stress, exercise (increased)
  • dehydration (increases)
  • overhydration (decreases)
97
Q

pathophysiologic classification of anemia

A
  • decreased RBC production (affecting stem cells or differentiating cells)
  • increased RBC destruction (hemolytic)
  • RBC loss (hemorrhage)
98
Q

morphologic classification of anemia

A

big - macrocytic
normal - normocytic
small - microcytic

99
Q

examples of decreased RBC production due to stem cell issues

A
  1. aplastic anemia
  2. pure red cell aplasia
  3. myelodysplastic syndromes
  4. anemia of renal failure
100
Q

examples of decreased RBC production due to differentiating cell issues

A 
impaired DNA synth
- B12 and folate
imparied hemoglobin synth
- iron deficiency
- thalassemia (globin chain defect)
- sideroblastic anema (heme synth defect)
multiple mechanisms
- anemia of chronic disease
- myelophthisic anemia - fibrosis
101
Q

examples of anemia caused by increased RBC destruction

A 
EXTRACORPUSCULAR
- immune-mediated
- non-immune-mediated
INTRACORPUSCULAR
- acquired
- inherited (RBC membrane defects, enzyme deficiencies, globin chain structural -sickle, or synthesis defects - thal)
102
Q

examples of macrocytic anemia causes (2)

A
  1. B12/folate deficiency

2. antineoplastic drugs

103
Q

exaples of normocytic anemia causes (5)

A
  1. aplastic anemia
  2. anemia of chronic disease (some)
  3. hemolytic anemia
  4. myelophthisic anemia
  5. acute blood loss
104
Q

examples of microcytic anemia causes (4)

A
  1. iron deficiency
  2. thalassemia
  3. sideroblastic anemia
  4. anemia of chronic disease (some)
105
Q

clinical general features of anemia (11)

A 
tissue hypoxia and
compensatory mechanisms
1. fatigue
2. dyspnea on exertion
3. faintness
4. vertigo
5. angina pectoris
6. claudication
7. palpitations
8. pallor
9. hypotension
10. tachycardia
11. systolic murmur
106
Q

clinical specific symptoms of anemia (7)

A
  1. GI or GU bleed (iron def anemia)
  2. PICA - eating clay or ice ( iron def anemia)
  3. JAUNDICE (hemolytic)
  4. DARK URINE (hemolytic)
  5. NEURO (B12)
  6. DRY ATROPHIC SKIN/NAILS (iron deficient)
  7. RED SWOLLEN TONGUE (B12)
107
Q

lab evaluation of anemia - step by step

A

ALWAYS DONE

  1. hematocrit, hemoglobin, RBC CBC
  2. RBC indices
  3. peripheral smear eval

SOMETIMES DONE
4. reticulocyte count

SPECIALIZED

  1. iron studies (microcytic)
  2. B12/folate (macrocytic)
  3. hemoglobin degradation products LDH (hemolytic)
  4. other tests for hemolytic anemias
  5. bone marrow
108
Q

MCV

A

Mean corpusular volume

  • measure of size
  • lower in kids than adults
109
Q

RDW

A

RBC distribution width

  • measures how uniform the cell size is
  • high in iron deficiency, not in thalassemia
110
Q

MCH

A

Mean corpuscular hemoglobin

- average hemoglobin content (parallels MCV and MCHC)

111
Q

MCHC

A

mean corpuscular hemoglobin concentration

  • concentration of hemoglobin in RBC
  • often low in microcytic anemias

color - area of central pallor

112
Q

polychromasia

A

larger and bluer - just released from bone marrow

113
Q

poikilocytosis

A

variation in shape of RBC

114
Q

Codocyte/target cell

A

darker in center
shaped like cup
too much membrane not enough cytoplasm

115
Q

teardrop/dacryocytes

A

myelofibrosis
metastatic
myelophthisic

116
Q

schistocytes/helmet cells

A

damaged in circulation

fibrin in DIC causes slicing of cell

117
Q

sickle/drepanocytes

A

sickle cell

118
Q

ovalocytes/elliptocytes

A

rare

hereditary ovalocytosis

119
Q

spherocytes

A

bits and peices removed, form sphere
no area of central pallor
hereditary spherocytosis
autoimmune hemolytic anemia

120
Q

burr cell/echinocytes

A

sometimes an artifact

see with uremic patients

121
Q

acanthocytes

A

thorn cells

122
Q

reticulocyte count

A

% = raw retic % x (pt hematocrit/mean normal hematocrit)

123
Q

TIBC

A

Total iron binding
capacity of serum to bind iron
reflection of serum transferrin levels

124
Q

serum ferritin

A

how much iron is actually in storage

125
Q

serum vit B12

A

for megaloblastic anemias, good reflection of vitb12 stores

126
Q

serum and RBC folate

A

for megaloblastic anemia,

serum is good measure, but labile. RB level better for total body stores, but subject to analytical problems

127
Q

intrinsic factor blocking antibody

A

megaloblastic

-proves cause of B12 deficiency

128
Q

tests for patients with megaloblastic/macrocytic anemia

A
  • serum vit B12
  • serum folate
  • IF blocking antibody
129
Q

hemolytic anemia tests

A
  • LDH
  • and hemoglobin degradation product (high unconjugated bilirubin, hemosiderin, low haptoglobin etc., hemoglobin in urine)
130
Q

coombs test

A

for autoimmune - antigolbuin, hemolytic anemia

131
Q

3 layers of immune defense

A
  1. barriers (skin, pH, enzymes)
  2. innate (neutrophils, macrophages, dendritic cells, NK cells - specialized lymphocytes)
  3. adaptive (T cells, B cell lymphocytes)
132
Q

innate and adaptive immune response communication/feedback

A

innate positively regulates/feedsback on adaptive immunity, adaptive regulates innate - don’t want ongoing damage due to inflammation. innate contains, adaptive clears

133
Q

receptors for innate immunity arise from what?

A

germline - they’re germline encoded - passed down from parents. are broadly specific (recognize broad molecular patterns associated with pathogens or tissue damage)

134
Q

receptor for adaptive immunity arise from what?

A

NOT germline encoded - antigen receptors require somatic recombination during development

135
Q

example of antimicrobial protein

A

defensins (seen in skin, gut, lungs, ENT) - can insert themselves into cell membrane of bacteria, fungi and some viruses (secretion can be induced by gut flora or epithelia or neutrophils)

136
Q

microbial flora/ commensal bacteria role

A
  1. competes with pathogenic bacteria
  2. induces epithelial and immune cells in microenvironment to produce anti-microbial innate factors – immune equilibrium
137
Q

complement system - 3 pathways and 3 effects (include specific complement molecules associated with effects)

A

3 pathways:

1) classical (antigen:antibody)
2) lectin (lectin binds to pathogen)
3) alternative (pathogen surfaces)

C3 CONVERTASE goes from pathways to effects

All pathways result in
1) recruitment of inflammatory cells (Chemoattractants - also make BVs leaky “C5a, C3a”)

2) opsonization of pathogens (coats to make cells recognizable to phagocytes) “C3b”
3) killing of pathogens by inducing pores within membrane of invading pathogens – assembles a membrane-attack complex (MAC) to make pores - enough pores will degrade the integrity of pathogen membrane “C5b, C6, C7. C8. C9”

138
Q

complement - lectin pathway

A

initiated by mannose binding lectin - interacts with mannose-containing carbohydrate present on pathogens

139
Q

complement- alternative pathway

A

spontaneous activation at low levels, interacts with negatively charged features of pathogen cell walls

140
Q

complement - classical pathway

A

activated by antibody-mediated recognition of antigen

141
Q

How do innate immune cells sense changes in the microenvironment (5 ways)

A
  1. Pattern Recognition Receptors (PAMPs, DAMPs)
  2. Toll-like receptors
  3. NOD-like receptors
  4. Lectin receptors
  5. scavenger receptors
142
Q

PAMP

A

Pathogen-associated molecular pattern
- molecules associated with microbes - bacterial and fungal cell wall constituents, bacterial/viral nucleic acids, bacterial proteins (i.e., flagellin)

examples: peptidoglycans, lippopolysaccharides, flagellin, DNA/RNA

143
Q

DAMP

A

Danger-associated molecular pattern

  • molecules released by host cells when they’re damaged or during necrosis (not by healthy or apoptotic cells). include uric acid, ATP, stress induced proteins (heat shock proteins), nuclear proteins (HMGB1)
  • NOD and TLRs recognize both PAMPs and DAMPs
144
Q

Consequences of pattern recognition receptor signaling

A

PRR signaling events stimulate macrophages and neutrophils to secrete lipid mediators of inflammation (PROSTOGLANDINS/LEUKOTRIENES), CHEMOKINES (IL-8 or CXCL8) and CYTOKINES (IL-1, IL-6 and TNF-alpha) which cause local inflammation that includes swelling, heat and redness and pain.

145
Q

what is a consequence of swelling during local inflammation?

A

(increase in fluid bring in complement factors that kill invading organisms, and clotting factors that prevent organisms from spreading)

146
Q

What does IL-1beta do (6 things)

A

pro-inflammatory cytokine
locally (lymphocyte/liver):
- enhances responses
- production of IL-6 –> acute-phase protein secretion

147
Q

What does TNF-alpha do? (5)

A

pro-inflammatory cytokine
locally (works on vascular endothelium):
- increases vascular permeability, which leads to increased entry of IgG, complement, and cells to tissues and increased fluid drainage into lymph
- VCAM, E- and P- selectin expression

148
Q

What does IL-6 do?

A

pro-inflammatory cytokine
locally (lymphocyte/liver):
- enhances responses
- induces acute-phase protein production

149
Q

What does CXCL8 (IL-8) do?

A 
chemotactic factor recruits (works on phagocytes):
- neutrophils
- basophils
- T cells
to site of infection
150
Q

What does IL-12 do?

A

pro-inflammatory cytokine (works on naive T cells)

  • pro-inflammatory
  • diverts immune response to T H1
  • cytokine secretion
  • NK activation
151
Q

Toll-like Receptor (TLRs) - examples, signal transduction

A

family of PRRs that recognize microbial molecules

Some TLRs are plasma membrane bound, others are in endosomal compartments

TLR generate signal transduction cascate reulting in NF-kB dependent gene transcription - inducing production of pro-inflammatory cytokines (TNFalpha, IL-6, IL-12 etc.)

152
Q

NOD-like receptors (example, issues)

A

located in cytoplasm - recognize microbial patterns and induce cells to produce cytokines (via NF-kB also)

  • NOD defects seen in subset of Crohn’s disease
153
Q

Scavenger receptors

A

recognize phospholipids on microbial cell wall - promote phagocytosis and cytokine production

154
Q

Myeloid innate immune cells

A
  • macrophages
  • dendritic cells
  • neutrophils
  • eosinophils
  • basophils
  • mast cells
155
Q

Lymphoid innate immune cells

A
  • NK cells

- others that will be covered later…

156
Q

Macrophage effector functions (3)

A
  • constitutively active, stay on site
  • engulf and kill invading pathogens
  • release lipid mediators of inflammation (prostaglandins and leukotrienes)
  • release chemokines and cytokines to recruit leukocytes (first wave includes monocytes that differentiate into macrophages once in tissue, and phagocytic neutrophils PMNs) - high levels of cytokine release will cause sepsis
157
Q

M1 macrophages

A

“classically” activated macrophages - associated with Th1 type immune responses (IFNg pro-inflammatory) - in obsese adipose tissue

158
Q

M2 macrophages

A

associated with Th2 type immune responses (IL-4 allergic inflammation) – wound healing, tissue repair, in lead adipose tissue

159
Q

mechanism of neutrophil recruitment to injury

A

neutrophils are continually produced by bone marrow and released by cytokines and chemokines

recruitment:
1) cytokines released by macrophages (TNF-alpha, IL-1) upregulate expression of ADHESION MOLECULES on BV endothelium. leukocytes become attached to selectins, integrins (bind to ICAM) cause leukocyte to stop rolling and adhere to endothelium.

2) integrins and chemokine receptors signal DIAPEDESIS (extravasation) into tissues
3) extravasated leukocytes respond to chemokine gradient (i.e. IL-8 and complement for neutrophils) via interaction with chemokine receptors –> leukocytes move toward injury which has high concetration of chemokines

160
Q

classes of adhesion molecules that regulate neutrophil recruitment

A
  1. selectins (P and E selectin expression upregulated by addressins, expressed by neutrophil)
  2. integrins
  3. members of immunoglobulin supergene family - ICAM, VCAM
161
Q

Mechanism of neutrophil effector function

A

neutrophils express receptors that interact with microbial patterns and facilitate phagocytosis

reactive oxygen and nitrogen species are made by NADPH oxidase to destroy the endocytosed organisms - called RESPIRATORY/OXIDATIVE BURST

162
Q

Dendritic cells (where they are, what they do (3 things))

A

constituatively present in tissue and sense the microenvironment using pattern recognition receptors.

activation causes production of cytokines that promote INITIAL imflammatory immune response

migrate from tissue to lymph tissue - where they serve as antigen presenting cell for NAIVE T CELL ACTIVATION

respond to viral infection via PLASMACYTOID DENDRITIC CELLS

163
Q

plasmacytoid dendritic cell (function)

A

plumper in appearance. rare-ish. subset of dendritic cells produces TYPE 1 INTERFERONS (IFN-alpha, IFN-beta) in response to VIRAL INFECTIONS

164
Q

functions of type 1 interferons (3)

A

1) induce anti-viral state by bystander cells (stimulation of enzymes that block viral replication if bystander cell becomes infected)
2) activate virus infected cell to increase MHC class 1 proteins to promove interaction with cytotoxic T lymphocytes
3) activate NK cells to be more efficient at killing infected cells

165
Q

what is the role of NK cells in innate immunity? (2)

A

1) to recognize and kill pathogens residing in the cytoplasm of host cells - viruses especially via KAR and enzymes. –
macrophages release chemokines (from granules) that recruit circulating NK cells to infected tissues. keep the replication of viruses to minimum until adaptive immune responses are activated

2) NK cells also produce interferon gamma - regulate development of immune response

166
Q

NK cell receptors

A

NK cells recgonize infected host by using receptrs that notice changes in host cell surface proteins.

1) Killer Activating Receptors (KARs) recognie viral-associated molecules on surface as well as molecules indicative of cell stress
2) Killer Inhibitory Receptors (KIR) recognize MHC class 1 expression - does not kill normal cells, but may decrease during viral infection (so altered MHC will kill target cell)

167
Q

NK mechanism of action

A

Once NK receptors are activated, cell resleases PERFORIN, GRANZYMES and GRANULYSIN which are stored in preformed granules - trigger caspases.

perforin forms pore in targe cell membrane
granzymes (trigger caspases) are released into cytopasm to initiate apoptosis. granulysin is antimicrobial and induces apoptosis

168
Q

how are NK and cytotoxic T cell actions different

A

similar mechanism and molecules, but NK cells don’t express T cell antigen receptor

169
Q

NALPs

A
  • NALPs (NOD-like receptor with pyrin-like domains) are important for IL-1beta (inflammasome activates caspase 1, which cleaves IL-1beta to be secreted). NALP associates with other molecules, forming macromolecular complex called INFLAMMASOME. - responds to microbe and DAMPs like URIC ACID IN GOUT.
  • familial periodic fever syndromes (familial mediterranean fever) mutations in pyrin
170
Q

effect of inflammatory cytokines on liver

A

causes:
acute-phase proteins (c-reactive protein, mannose-binding lectin)

which in turn causes:

  • activation of complement
  • opsonization
171
Q

effect of inflammatory cytokines on bone marrow

A

causes:
neutrophil mobilization

which causes:
- phagocytosis

172
Q

effect of inflammatory cytokines on hypothalmus

A

causes
increased body temp

which causes:
decreased viral/bacterial replication

173
Q

effect of inflammatory cytokines on fat/muscle

A

causes:
protein/energy mobilization to increase body temp

which causes: decreased viral/bacterial replication

174
Q

2 examples of stimuli that regulate movement of dendritic cells into lymph nodes

A
  • PAMP

- chemokine

175
Q

someone with decreased beta-2 integrins (CD11a or LFA-1) would present with what?

A

increased circulating neutrophils - can’t get into tissue - leukocytosis

176
Q

describe graph of immune response events after infection (levels of substances you would see in the blood)

A

1) pro-inflammatory cytokines are first (~2 days after infection) - IFN-alpha, beta (made by dendritic cells - IFN-1s), TNF-alpha, IL-12
2) NK mediated killing of affected cells - contain virus
3) T-cell (adaptive) mediated killing of infected cells - eliminate virus

177
Q

Step by step, what happens when you get a splinter

A
  1. splinter happens, damages cells (releases DAMPS) introduces bacteria (microbial- associated microbial patterns - PAMPs)
  2. constitutively resident macrophages and dendritic cells have pattern recognition receptors that respond to DAMPs and PAMPs
  3. macrophages and dendritic cells are activated and produce cytokines and chemokines to recruit additional cells to contain infection
  4. adaptive immunity comes later
178
Q

possibilities when you see microcytic anemia

A

1) iron deficiency
2) anemia of chronic disease
3) thalassemias (microcytosis out of proportion to hb/hematocrit)

179
Q

iron deficiency anemia initial differential (after obtaining CBC)

A

In CBC, when you see low Hct, RBC, Hb, MCHC, MCH and MCV, and high RDW and platelets, think iron deficiency.
Iron deficiency anemia is always due to an underlying cause:

1) suspect bleed (GI or GU bleed should always be suspected first in older man and post-menopausal woman)
2) decreased uptake (inadequate diet, gastric surgery - Fe absorbed in proximal jejunum, celiac disease, increased gastric pH, tannins in tea)
3) increased requirements - pregnancy, childhood

180
Q

clinical manifestations of iron deficiency

A
  • symptoms of anemia (fatigue, shortness of breath, dizziness, palpitations)
  • PICA - eating random shit (ice, clay, chalk)
  • restless leg
  • KOILONYCHIA - spooning of nails
  • blood in stool (if GI bleed is cause) or urine (if GU bleed is cause)
181
Q

additional lab tests for iron deficiency anemia

A 
- iron panel (serum iron, serum ferritin, TIBC, sTFR)
see:
- decreased serum iron
- decreased iron saturation
- decreased serum ferritin 
- increased total iron-binding capacity
182
Q

treatment for iron deficiency anemia

A

treat underlying cause (malnutrition, GI bleed)

- give oral supplements or parenteral if oral isn’t tolerated

183
Q

macrocytic anemia subtype

A

megaloblastic (B12, folate)

non-megaloblastic (alcohol, liver disease)

184
Q

B12 deficiency - classic findings

A
  • megaloblastic anemia
  • can see pancytopenia (anemia, leukopenia and thrombocytopenia)
  • takes years to develop (as opposed to folate deficiencies which can develop quickly)
  • neurological changes (myelopathy of posterior columns - vibration and position sense, of lateral columns - weakness, spacticity)
  • low retic count
  • hypersegmented neutrophils
  • macro ovalocytes
  • see high methylmalonyl coa and homocysteine
185
Q

causes of B12 deficiency

A
  1. pernicious anemia (autoimmune, seen with other autoimmune defects, failure to secrete intrinsic factor because of antibody)
  2. surgical (gastrectomy or gastric bypass, resection of terminal ileum)
  3. zollinger-ellison syndrome - inactivation of pancreatic protease which impairs B12 binding to iF
  4. pancreatic exocrine deficiency (enzymes)
  5. blind loop syndrome (colonizing bacteria bind B12)
  6. diphyllobothrium latum (worm that binds B12 and prevents absorption)
  7. dietary (vegans)
186
Q

treatment for B12 deficiency

A

intramuscular B12
oral B12
phrophylactic therapy for gastric/ileum resection
watch out for hypokalemia with treatment early on

187
Q

folic acid deficiency - classic findings

A
  • low serum folate
  • low RBC folate
  • elevated serum homocysteine level
188
Q

folic acid deficiency - causes

A
  • inadequate diet (mostly with elderly, poor and alcoholics)
  • impaired absorption (due to sx)
  • medications (anticonvulsants, contraceptives)
  • increased requirements (pregnancy, chronic hemolytic anemia, exfoliative dermatitis)
189
Q

anemia of chronic disease

A
  • usually normocytic, can eventually become microcytic
  • ## cytokine driven (iron unavailable - sequestered in macrophages)
190
Q

cytokine role in anemia (epo, hepcidin etc.)

A

?
cytokines:
suppress erythropoeisis and increase hepcidin production.
increased hepcidin increases iron sequenstration
renal failure decreases hepcidin excretion into urine, decreases epo
macrophages increase phagocytosis

191
Q

causes of ACD

A
  • acute/chronic infection (HIV, fungal, parasitic)
  • malignancy
  • autoimmune (lupus, inflammatory bowel disease)
  • hospitalization
  • end stage renal disease
192
Q

RDW in iron deficiency vs ACD

A

high in iron deficiency, normal in ACD

193
Q

platelet levels in iron deficiency vs ACD

A

can be normal to high in either

194
Q

wbc count in iron deficinency vs ACD

A

Normal to high in ACD, normal in iron deficiency

195
Q

treatment for ACD

A

treat underlying cause
transfuse
erythropoeitic stimulating agents

196
Q

general lab characteristics of hemolytic anemia (from CBC, marrow, smear)

A
  • high retic count (increased erythropoesis)
  • increased degradation products (LDH, bilirubin)
  • decreased haptoglobin
  • erythroid hyperplasia in marrow (increased RBC precursors)
  • polychromasia (blue-ish immature cells)
  • immature nucleated RBCs
  • schistocytes (microangiopathic)
  • microspherocytes (AIHA)
197
Q

clinical features (physical presentation) of hemolytic anemia (both intra and extravascular)

A
  • general anemia symptoms
  • jaundice

iF INTRAVASCULAR
- can see hemoglobinemia, hemoblobinuria, shock, DIC, hypotension, renal failure

IF EXTRAVASCULAR (TBCs destroyed in phagocytosis - can see with sickle cell)

  • splenomeglay
  • gall stones
198
Q

hereditary causes of hemolytic anemias (8)

A
  • G6PD deficiency
  • pyruvate kinase deficiency
  • heridtary spherocytosis
  • hereditary ovalocytosis
  • sickle cell
  • HbC
  • HbE
  • Thalassemia
199
Q

acquired causes of hemolytic anemia

A
  • immune hemolytic anemia (AIHA)
  • mechanical (shear stress)
  • microangiopathic (DIC, TTP)
  • Infection (malaria, clostridium)
200
Q

How to diagnose immune hemolytic anemia

A

Coombs test (direct tests for antigens on RBCs for IgG or complement)

WARM: IgG (spleen)
COLD: IgM - fixes complement (liver and spleen)

201
Q

Warm AIHA - associated disorders

A
  • idiopathic
  • drugs (methyl dopa, penicillin)
  • lymphoid malignancies
  • autoimmune (SLE)
202
Q

Cold AIHA - associated disorders

A
  • idiopathic (cold agglutinin disease)
  • lymphoid malignancies
  • infections - ebstein barr, mycoplasma
203
Q

AIHA clinicla presentation (6)

A
  • anemia
  • jaundice
  • cardiopulmonary collapse
  • hepatosplenomegaly
  • thrombosis
  • acrocyanosis (cold agglutinins)
204
Q

Warm AIHA treatment

A
  • treat underlying (discontinue drug if drug-induced)
  • immune suppression to decrease antibody production
  • splenectomy (warm) -site of destruction
205
Q

Cold AIHA treatment

A
  • treat underlying (EBV etc.)
  • administer warmed RBCs
  • plasma exchange if really severe
  • avoid cold
206
Q

G6PD deficiency anemia (genetics)

A
  • x-linked disorder
  • A form in african american men (milder)
  • Mediterannean type more severe (even reticulocytes have low levels of G6PD)
207
Q

G6PD hemolytic triggers

A
  • increased susceptibility to oxidative damage
  • infection or medication (antimalarials, sulfonamides) cause oxidative stress to trigger anemia
  • fava beans also increase oxidative stress
208
Q

lab tests for G6PD hemolytic anemia

A
  • Heinz bodies and bite cells

- G6PD levels in older RBCs

209
Q

hereditary spherocytosis (genetics)

A
  • autosomal dominant

- defective ankyrin protein

210
Q

hereditary spherocytosis (clinical manifestations)

A
  • chronic anemia
  • splenomegaly
  • gall stones
211
Q

hereditary spherocytosis (lab tests)

A
  • see spherocytes
  • increased osmotic fragility
  • hemolytic anemia findings (LDH, breakdown products, jaundice, high retic, low haptoglobin)
212
Q

treatment for hereditary spherocytosis

A
  • splenectomy in severe cases
213
Q

2,3 BPG and oxygen shift

A

2,3, BPG decreases O2 affinity - right shift

214
Q

alkylosis and oxygen shift

A

alkylosis causes increased O2 affinity - left shift

215
Q

genetic differences between beta thal major, minor and intermedia

A

all beta thal has impaired beta chain synthesis
minor = heterozygote
major (B0) = homozygote
intermedia (B+) = homozygote but only impaired, not deleted

216
Q

consequences of beta thal defects in beta thal major

A

alpha chain preceipitates cause:

1) ineffective erythropoesis
2) hemolysis
3) marrow expansion (in face - chipmunk)
4) extramedullary hematopoesis (spleen, spine)
5) high output congestive failure

217
Q

electrophoresis and thalassemias (what can you use it for)

A

can determine the type of beta thalassemia (see if you have delta, beta or gamma) - alpha doesn’t work - always the same percentage (have to use PCR or gene sequencing)

218
Q

treatment for beta thal major

A
  • transfusion (but be careful about iron overload)
  • hydroxyurea (increases fetal hemoglobin)
  • stem cell transplantation
219
Q

what do you expect to see in a beta thal minor patient’s CBC - RBC, Hgb, Hct, MCV

A

you would see a disproportionally high RBC to Hgb and Hct (could all be low, but RBC is less low). see low MCV (disproportionatly low as compared to Hct)

220
Q

how many loci can have mutations in alpha thal

A

4 total. 1, 2 or 3 are viable with life, 4 isn’t (Bart’s Hydrops Fetalis - no functional alpha globin genes - all gamma, leads to eclampsia and stillbirth)
3 defects lead to hemoglobin H (beta chains combine with each other, causes serious fast hemolysis - need transfusion therapy)

221
Q

sickle cell treatment

A 
prophylactic antibiotics until age 5 
pneumococcal vaccine for kids
hydroxyurea 
oxygenation 
transfusion (simple or exchange)
222
Q

why do you get gall stones in sickle cell

A

you have increased red cell destruction, increased bilirubin - gall stones

223
Q

why do you get necrosis of bone structure in sickle cell

A

can’t get O2 to bone structure because of sickled shape

224
Q

complications of sickle cell

A
  • acute chest
  • stroke in kids
  • leg ulcers in adults
  • seizures
  • renal failure
  • bone necrosis

I3 (2 decks)

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