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Hematology Flashcards

1
Q

Microcytic anemias -
Lab(s) to order
Production or destruction?
5 main types

A

Hgb < 13 —> look at MCV …<80 = microcytic

Order reticulocyte count - microcytic anemias are usually production issues = LOW (< 0.5%) reticulocytes (b/c don’t have correct ingredients)

THEN order:
FERRITIN (most important), Fe level, TIBC

Main types: 
Iron deficiency 
Thalassemia 
Anemia of chronic disease 
Sideroblastic anemia
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2
Q

Normocytic anemias -
Labs to order
Usually production or destruction?
4 main types

A

Hgb < 13, MCV 80-100, RI > 2% (destruction)

Labs: Hemolysis labs = LDH, haptoglobin, T.bili/D.bili –> if POSITIVE for hemolysis, expect HIGH LDH, LOW haptoglobin, HIGH T.bili then that is the cause of the anemia

Usually almost always destruction (HIGH RI > 2%) b/c bone marrow trying to keep up with losses via HEMORRHAGE or HEMOLYSIS

Exception: Can be normocytic anemia w/ LOW RI (production issue) if person has leukemia, myelodysplastic syndrome, or CKD (no EPO)

If test asks you ONE test to order, order a reticulocyte index or count

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3
Q

Macrocytic anemias -
Labs to order
Usually production or destruction?
3 main types

A

PRODUCTION issue (LOW RI %)

Labs to order - BLOOD SMEAR - megaloblastic (5+ lobes) or non-megaloblastic PMNs?

Megaloblastic - Low B12 or low folate - both low or equivoccal order methylmolonic acid - HIGH in B12 def, normal in folate def

Non-megaloblastic - ETOH, liver dz, drugs, metabolic issues etc

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4
Q

Labs consistent with Fe-def anemia

A

MCV < 80 (microcytic)

Patho: deficiency in Iron - SLOW bleeds b/c bone marrow keeps up - but uses up all of its iron stores along the way

Ferritin = LOW
Fe = normal to low
TIBC = HIGH

Tx: 324 mg Iron TID w/ stool softener

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5
Q

Labs consistent w/ anemia of chronic disease

Tx?

A

Patho: Inflammation - cells sequester iron from bacteria b/c they can’t survive without it - chronic inflam dz causes the same

Ferritin = HIGH (also elevated b/c ferritin = an inflammatory mediator & there’s inflammation)

Fe = LOW (disconnected from silo) 
TIBC = LOW

Tx: NOTHING. Treat underlying inflammation/condition rather than go after the anemia. Can use EPO in severe severe cases.

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6
Q

Two classic patients with Iron-def anemia

A

Young woman w/ menorrhagia

MAN > 50 YO w/ FOBT+ = Colorectal cancer!!!

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7
Q

Type of patient you would see anemia of chronic disease in? Are they usually symptomatic or asx?

A

SLE, RA etc - any person with chronic inflam dz so AI ones are common

Usually ASX

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8
Q

Two types of patients with thalassemia

A

Minor pt - asx

Transfusion-dependent pt

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9
Q

Labs in thalassemia

A

Hgb < 13 (anemic)
MCV < 80 (microcytic)

Order iron studies:
Iron = NORMAL
Ferritin = NORMAL
TIBC = NORMAL

Iron studies are NORMAL because it is an issue with the chains of hemoglobin not the actual heme part

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10
Q

How many genes make up alpha hemoglobin? Beta hemoglobin? When do each become symptomatic?

A

1 alpha deleted - ASX
2 alpha deleted - mild sx
3 alpha deleted - SEVERE sx
4 alpha deleted - DEAD

1 beta deleted - mild sx/dz
2 beta deleted - severe sx/dz

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11
Q

What do you order when you see a microcytic anemia with normal iron studies?

A

HEMOGLOBIN ELECTROPHORESIS!!!!

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12
Q

Hgb electrophoresis results if person has beta thalassemia?

A

Normal Hgb is two alpha chains and two beta chains = HbA1

IF you don’t have enough beta chains (b/c one deleted)- there will be too many alpha chains and it will pair up with something else - fetal hemoglobin (HgF) or other HgA2

SO…if you have a problem with BETA, you’ll see other combos - shows you definitively Beta thalassemia = beta thalassemia dx right there

BETA = FETA - happens in greeks

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13
Q

Microcytic anemia

Normal Iron studies

Normal Hgb electrophoresis

A

Alpha thalassemia

Explanation: B chains can only pair with alpha chains - so whatever B chains there are will pair with alpha & Hgb electrophopresis will appear NORMAL & therefore you get alpha thalassemia as a dx of exclusion - you didn’t see the alpha making other odd matchups b/c was b-deficient so that means you’re a-chain deficient

Word NormAl has an A in it- normal Hgb electrophoresis = ALPHA thalassemia!

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14
Q

Tx mild alpha or beta thalassemia

A

ASX, nothing

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15
Q

Tx severe alpha or beta thalassemia

A

Transfusions monthly - each one = 324 G IRON (yearly supply) - so will become iron-overloaded

Treat w/ DE-FUROX-AMINE (get iron out of me)

NOT PHLEBOTOMY - that would make their anemia WORSE DUH

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16
Q

Sideroblastic anemia labs

A

Patho: Iron gets stuck in mitochondria so marrow throws more iron at them - cells themselves are loaded w/ iron - just can’t do anything so measure blood & Fe elevated

Elevated Fe
Other studies don’t matter

Or picture of bx that shows RING SIDEROBLASTS - RBC W/ DARCK CIRCLE IN IT

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17
Q

Reversible causes sideroblastic anemia

A

Drugs
ETOH
LEAD

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18
Q

Irreversible causes sideroblastic anemia

A

B6 deficiency - CANNOT CORRECT W/ B6

Myelodysplastic syndrome

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19
Q

Labs consistent with anemia 2/2 hemolysis? What to order if labs show hemolsysis?

A

Hgb < 13

MCV 80-100 (normal)

RI > 2% (something destructing cells, bone marrow kicks in to pump out more)

+ Hemolysis:
LDH - HIGH
Haptoglobin - LOW
T. BILI - HIGH

ORDER A SMEAR IF + FOR HEMOLYSIS…..

G6PD -bite cells, heinz bodies

Sickle cell - sickled cells

Hereditary spherocytosis- spherocytes

AI hemolytic anemia - also spherocytes

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20
Q

If normocytic anemia w/ RI> 2% and hemolysis labs negative? What is the most likely cause?

A

HEMORRHAGE

PLUG HOLE, GIVE BLOOD

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21
Q

Sickle cell - patho, when crisis occurs, how crisis manifests

A

Patho: Autosomal

When crisis occurs: Vaso-occlusive crisis 2/2 acidosis, hypoxemia, dehydration –> Cells sickle & cannot get thru capillary & two things occur - hemolysis or capillary becomes obstructed & tissue becomes ischemic (= PAIN)

CP: Acute chest (MI, CHF), acute brain (CVA), priapism

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22
Q

Original dx of sickle cell

A

Hemoglobin electrophoresis - once diagnosed

Do smear for all others

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23
Q

Chronic sickle cell treatment

A

Hydroxyurea - induces production of hemoglobin F (fetal) = gene which is okay so you get Alpha2F2

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24
Q

Acute sickle cell treatment

A

IVF
O2
Pain control
Tx underlying condition (infection)

BUT in setting of acute chest, acute brain, priapism - do exchange transfusion - take out bad blood & put in good blood

AVOID REGULAR blood transufusions b/c will become Iron-overloaded - don’t transfuse based on numbers (nl hgb for sickle cell pt is 7-8), transfuse based on SYMPTOMS

If do transfuse blood, treat Fe overload w/ defuroxamine (undo the iron from me)

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25
Q

Vaso-occlusive crisis in young person look for…?

A

Sickled cells

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26
Q

G6PD deficiency - Patho, epi

A

Patho: Genetic mutation, in AA & mediterannean = dec G6PD (usually repels oxidative stress) so in cell deficient in G6PD, oxidative stress comes thru & destroys the cell

AA man exposed to medication w/ oxidative stress –> DAPSONE, BACTRIM, nitrofurantoin –> otherwise totally normal (no hemolysis at baseline) - don’t need to do anything here

OR Greek person exposed to fava beans & can be FATAL. Always have chronic low level hemolysis. Exposure –> TURN YELLOW

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27
Q

Dx G6PD

A

First: Peripheral smear - heinz bodies, bite cells

Best: G6PD level - DO NOT GET A G6PD LEVEL DURING AN ATTACK - will be artificially normal - get 6-8 weeks after

Tx: Supportive during attack, avoid oxidative stress

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28
Q

Hereditary spherocytosis - patho, CP, Tx

A

Patho: RBC membrane protein defects = cells are spheres & not donuts - spleen says hey you can’t fit thru me, you must be bad & lyses them

Dx: First test will probs be peripheral smear - spherocytes (not specific) so BEST test = osmotic fragility - confirms dx

Pt: Presents w/ hemolysis & spherocytes (not characteristic hx - generally a labaratory dx)

Tx: Splenectomy - won’t change dz, but prevents anemia - if no splenectomy need Fe and folate supplementation

NEGATIVE COOMBS!!! AIHA has positive coombs –> How you tell them apart b/c they can both have spherocytes

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29
Q

Autoimmune hemolytic anemia - order what for dx? Cold vs hot antibody?

A

AIHA = order coombs test, will be POSITIVE for cold and hot AIHA

COLD AIHA - Coombs + for IgM, caused by mycoplasma, mono –> tx = avoid cold

HOT AIHA - Coombs + for IgG - a/w AI disease, CA –> tx = fix underlying condition –> but that’s hard to do so use steroids, ritixumab, or splenectomy

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30
Q

Paroxysmal nocturnal hemoglobinuria - patho, CP, dx, TX

A

Patho:
Deficiency of PIG-A gene –> increased risk complement can fix b/c of missing anchor protecting cell & MAC complex forms, cell gets lysed

CP:
Paroxysmal (only happens in certain conditions) nocturnal (sleep don’t breath as often/deeply, get hypoxemia, acidosis = more cell lysis/complement) = hemoglobinuria = DARK URINE (looks like peeing blood but actually = Hgb)

Dx:
Flow cytometry - shows monoclonal expansion of CD55 negative cells

Tx:
Usually nothing
Severe/refractory - eculizumab

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31
Q

Macrocytic anemia - labs to order, diseases

A

Si/sx anemia - SOB, DOE, syncope, fatigue, malaise

Labs - Hgb low, MCV > 100, Reticulocyte count - LOW (production issue)

Order - peripheral smear - 5+ lobes = megaloblastic, <5 lobes = non-megaloblastic

Macrocytosis and megaloblastosis are NOT the same thing - macro= big megalo = impaired DNA synthesis - no impaired DNA syn = no hypersegmented neutrophils = non-megaloblastic anemia

Megaloblastic = B12, folate deficiency

Non-megaloblastic = liver failure, ETOH etc

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32
Q

What is a megaloblastic anemia, how do you diagnose it (labs)?

A

Si/sx anemia, or found on CBC - low Hgb, MCV > 100 = macrocytic

Ordered a smear - shows hypersetmented neutrophils = MEGALOBLASTIC aneima

2/2 B12/folate deficiency - order levels - if B12 low, repace w/ B12. If folate is low, supplement folate.

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33
Q

What do you do if you discover a megaloblastic anemia, order a B12/folate to see which it is caused by and the levels are equivocal?

A

Order a methylmolanic acid - it is elevated in B12 deficiency, and normal in folate deficiency

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34
Q

Causes of non-megaloblastic anemia

A

Liver disease - cirrhosis
ETOH
Drugs (chemo agents)
Metabolic diseases

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35
Q

Where does folate come from? How long of a supply can our body store at once? Type of patient with folate deficiency? Tx if folate is low?

A

Patho: Folate deficiency - comes from leafy greens - we can only store a 3-6 week supply - therefore must eat continuously

Pt w/ deficiency:
TEA AND TOAST DIET - chronic malnourished alcoholic - little old lady eating little packaged food at home

Tx: Folic acid 1 mg PO daily

If equivocal - order a methyl molonic acid - will be normal in folate def, elevated in B12 def

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36
Q

Causes of B12 deficiency

Sources of B12

How long of a supply of B12 can our body store?

Why do we care about B12 deficiency?

A 
Causes: 
Strict vegan 
Pernicious anemia
Chron's disease
Gastric bypass

Sources:
Animal products

Body Store:
3-10 years supply at once…aka you’re not going to run out unless you’re a strict idiotic (not supplementing) vegan for 10+ years

Why we care:
B12 deficiency causes neurological deficits that are PERMANENT once they onset (cord degeneration)

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37
Q

Dx & Tx B12

A

Dx:
Hgb<13, MCV>100, 5+ lobes on smear, B12 low

**If B12 equivocal, order MMA, if HIGH then B12 deficiency

Tx: Supplement B12 either PO or IM

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38
Q

How is vitamin B12 absorbed?

A

Parietal cells secrete intrinsic factor, you ingest B12. IF binds B12, goes to terminal ileum

Therefore you must have an intact terminal ileum AND an intact stomach to absorb B12

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39
Q

Why do Chorn’s patients and gastric bypass patients end up with B12 deficiency?

What kind of B12 replacement do these patients need?

A

The MC site of inflammation in Chron’s is terminal ileum or resected 2/2 fistula there means you cannot absorb B12 & IF

You’ve lost your absorption site!

Gastric bypass pt - end up with B12 def b/c lose parietal cells so don’t secrete IF so can’t absorb even w/ intact terminal ileum

They need IM B12 because they have impaired absorption so if you gave it back orally they couldn’t absorb it

If pt is just nutritionally deficient, can replace orally

40
Q

Patho of B12 neurological sx, clinical deficits? Looks like?

A

B12 deficiency = degeneration & demyelination of the dorsal column of the spinal cord = BAD –> once you lose them you can’t get it back

Responsible for two point discrimination, proprioception, & vibratory sense

LOOKS LIKE NEUROSYPHILIS

Running into things (loss of proprioception), flopping feet

Can feel pain & move - just clumsily

41
Q

How to interpret shillings test? What does it tell you? How to give?

A

Tells you impaired absorption vs impaired intake

Give them B12 IM (saturate liver) - then give oral B12

Interpretation: Check urine - b/c liver saturated, any B12 absorbed at this point will be peed out - SO If have B12 in their pee, then they can absorb & were just nutritionally deficient

If no B12 in urine = absorption problem…must give IM B12

DO NOT PICK THE SHILLINGS TEST ATS NEXT STEP BUT May be asked to interpret it

42
Q

What does leukemia mean?

A

WBC cancer (leuk)

In the blood (emia)

43
Q

Chronic leukemias =

What kind of cell?

WBC count? Diff?

How does the pt present?

Next steps if you’re suspicious of CML or CLL?

A

Chronic - mature WBC cell - b/c it’s chronic you can have a TON of them & they don’t reek havoc - just cells that should’ve died that haven’t

Therefore chronic pt presents ASX - incidentally found SEVERELY elevated WBC (>60)

CBC differential - will give you PMNs (“polys”) = MYELOGENOUS leukemia

OR

Lymphocytes = Chronic LYMPHOCYTIC leukemia

Next step = BONE MARROW BX

Chronic leukemias present insidiously

44
Q

Treatment CML

A

Imatinib

45
Q

Treatment CLL

A

HSCT if young

46
Q

ACUTE leukemias present with?

What kind of cell?

WBC? Diff?

A

Acute in leukemias is synonymous with BLAST CELLS - BM going CRAZY and REEKING HAVOC

Clinical presentation: Bone marrow going crazy, crowds out all other cells = so NO WBC, no RBC, NO plt = INFECTIONS, ANEMIC, BLEEDING

GET SMEAR - will see BLASTS!!! - if PMN = AML - if lymphocyte blasts = ALL

GET BONE MARROW BX to confirm dx of these cancers

47
Q

AML M3 variant tx

A

Vitamin A (alltrans-retinoic acid)

48
Q

AML tx - all variants other than M3

A

Chemotherapy

49
Q

BMB w/ > 20% PMN blasts = which kind of cancer?

A

AML

BLASTS = ACUTE
PMNs = myelogenous (MYELOID = NEUTROPHILS, M=N) leukemia  =

AML

50
Q

BMB w/ > 20% lymphocytes blasts = which kind of cancer?

A

ACUTE (blasts)

Lymphocytes = lymphocytic leukemia =

ALL

51
Q

ALL treatment

A

Chemo

PPX CNS

52
Q

What to order if you suspect a leukemia based on a CBC w/ dif?

A

Bone marrow biopsy

53
Q

AML typical patient, causes, dx,

A

Pt presents ACUTELY - OLD MAN - remember blasts are FUCKING up the bone marrow = infection, fever, anemia, bleed, bone pain etc

Caused by : exposure to benzine or radiation, or CML undergoing blast crisis

Dx: Smear > 20% blasts or BMBx > 20% blasts (PMNs, remember myeloid = neutrophils = aMl)
+ MYELO-peroxidase

54
Q

Auer rods are associated with?

A

AML - M3 type

Treated with Vitamin A

SO if you see a picture of a blast cell (looks fucked up) look for Auer rods (look like little splinters in side cell) = AML M3 subtype!!!

55
Q

ALL

Pathology, cell type, presents like? Biomarkers?

A

ACUTE PRESENTATION b/c blasts are proliferating and fucking up bone marrow - all cell lines down = infection, fever, anemia, bleeding, bone pain

Dx:
Blood smear > 20% blasts
BMBx > 20% lymph blasts Biomarkers - cALLa, TDT

Tx:
Check CNS - PPX CNS w/ chemo if negative (chemo = (ARA-C)) & generalized chemo

If find CA in CNS, give radiation

56
Q

AML average age?

ALL average age?

A

AML = OLD MAN (>60)

ALL = LITTLE kid (7 YO) = PEDIATRICS DISEASE

57
Q

ALL biomarkers

A

cALLA

TDT

58
Q

Chronic Myeloid leukemia

Typical pt
Presentation
Dx
Tx

A

Chronic = indolent presentation, myeloid = neutrophils (PMNs), luekemia = of the blood

Typical Pt = MIDDLE-AGED, ASX

Dx:
CBC –> TONS OF CHRONIC CELLS that haven’t died(WBC>60,000)

BMBx = confirms - looks for philadelphia chromosome = BCR-ABL = 9:22 translocation

Tx: Imatinib

59
Q

Chronic lymphocytic leukemia

A

Chronic = indolent presentation, lymphocytic = lymphocytes

CP:
OLD, ASYPTOMATIC

Dx:
First w/ CBC w/ diff - shows tons of lympohcytes, TONS of chronic cells that haven’t died - then get a bone marrow biopsy

Tx: NOTHING if they’re old & ASX - CaLL your grandma (she’s got CLL, but will die with it, not from it)

Symptomatic (from blood hyperviscosity)- = headache, bleeding –> THEN CHEMO

If YOUNG & donor, then give stem cell transplant

60
Q

What happens when CML targeted therapy drugs fail?

A

When imatinib & newer tyrosine kinase inhibitors fail, have BLAST crisis = CML turning into AML

61
Q

AUER RODS and myeloperoxidase positive

A

AML

Auer rods = AML (U looks like hump of an M)

Type that can be treated w/ vitamin A!

62
Q

Lympocytic leukemias ages of presentation?

A

ALL = KIDS - want to save ALL the children

CLL = OLD (80s) - CaLL your grandma

63
Q

Causes of low platelets

A 
Alphabet soup! 
ITP
TTP
DIC
HIT
64
Q

TTP
Patho
Presentation
Labs

A

TTP = thrombotic thrombocytopenic purpura

Patho:
Formation of microangiopathic hyaline clots which SHRED up RBCs & platelets& forming clots obstructing flow to organs (= renal failure, neuro sx)

Present: FATRN
Fever
Anemia (microangiopathic hemolytic variety)
Thrombocytopenia
Renal failure
Neuro sx (stroke)

CBC w/ smear:
LOW PLATELETS
Shistocytes (chewed up RBC)

DIC PANEL - normal (get to rule out DIC)
PT/aPTT - normal
Fibrinogen - normal
D-dimer - normal

65
Q

What do you order if you have someone who presents febrile and anemic with low platelets?

Next lab to order?
Treatment?

A

Low plt = thrombocytopenia…think of causes (alphabet soup…TTP, ITP, DIC, HIT…)

The pt also has fever and low RBC….so RBC getting chewed up, have FAT (fever, anemia, thrombocytopenia) of FATRN dx of TTP

Next step = order DIC panel to rule out DIC = PT/aPTT, fibrinogen, d-dimer –> all normal (b/c microangiopathic clot NOT a fibrin clot…DIC = fibrin clot)

Next step = exchange transfusion (take out bad blood, put in good)

NEVER TRANSFUSE PLATELETS

66
Q 
DIC 
Patho
Presentation 
Dx
Labs
A

Patho - problem w/ fibrin clot - caused when the person is SICK AS SHIT & their body goes WHACKO –> body starts forming clots everywhere it shouldn’t = all plt & clotting factors used up everywhere else

Presentation:
SEPSIS, ICU, SHOCK –> leads to DIC

Dx:
SICK AS SHIT - any pt who is super sick can go into DIC –> anyone who is SUPER sick & BLEEDING = DIC = must suspect

Labs:
PT/PTT = HIGH (clotting factors used up in clots)
Fibrin = LOW (used up)
D-dimer = HIGH

Tx: Supportive (transfuse whatever they don’t have) & FIX underlying disease (reverse shock/sepsis that tipped all of this off)

67
Q

HIT = heparin-induced thrombocytopenia
Patho
Typical pt

A

Patho: Ab to platelets caused by heparin = clot where they shouldn’t & bleed where they need clots

Typical pt:
In hospital on HEPARIN product, 7-14 days after initiation, the patient’s platelets PLUMMET

Dx:
> 50% drop in plt suddenly, +

Tx:
STOP HEPARIN
Start argatroban
Bridge to coumadin or warfarin

68
Q

ITP

Patho
Dx
Typical pt /hx

A

Immune thrombocytopenic purpura

Patho: Ab to platelets

Hx: WOMAN W/ AUTOIMMUNE DISORDER & low platelets

Dx:
Diagnosis OF EXCLUSION - look for correct history & RULE OUT ALL OTHER FORMS OF thrombocytopenia (HIT, DIC, TTP)

Tx:
Acute flare - steroids
Critically low = IVIG (hide plt from ab so they don’t die)
Refractory dz - splenectomy is curative

Fail splenectomy - rescue therapy is with ritixumab

69
Q

Lymphoma = ?

MC Presentation?

LN characteristics?

A

Cancer of the LN

Non-tender LAD - probably going to be cancer…fixed, firm = CANCER

Reactive LN (from infectious causes) HURT & they are freely mobile

ESPECIALLY IF YOU HAVE B SYMPTOMS –> upstages the cancer - about staging NOT diagnosis - upstages it from A to B

70
Q

Nontender fixed firm LN…next step?

A

Excisional biopsy

FN IS NOT GOOD ENOUGH - NEED TO CUT OUT LN & FLAY IT OPEN TO SEE CYTO-ARCHITECTURE & you can also do staining and smears - can be reactive & supposed to be tender so before you throw it away, do gram stain, AFB, fungal

ANY NON-TENDER LAD
—> CUT IT OUT!!!!

71
Q

4 possibilities from excisional biopsy of a LN

A
  1. Other cancer mets to LN - look for primary cancer
  2. Hodgkins Lymphoma = REED STERNBERG CELLS - RSH - reed sternberg hodgkins
  3. Non-Hodgkins Lymphoma
  4. Negative - ? reactive LN that was non-tender ….do gram stains to make sure not infected before throwing out
72
Q

Staging for NHL/HL

A

PET CT

Old way - CXR (if negative)—> then CT chest/ab/pelvis (if negative), then BMBx (positive = metastatic)

73
Q

Tx lympohma

A

Systemic chemotherapy for all forms/stages

74
Q

Staging lympohma

A

Stage 1 = 1 LN same side

Stage 2 - > 2 LN same

Stage 3 = > 2 LN opposite side

Stage 4 = metastatic

75
Q

Hodgkins or NHL better prognosis?

A

Hodgkins = MUCH BETTER - can cure

NHL = NOT SO GOOD - will die eventually from a lymphoma-related illness

76
Q

Hodgkins presentation vs Non-Hodgkin’s

A

Hodgkins = + B symptoms - night sweats, fever, weight loss. Presents in stage IIa or better, spreads contiguously (only in area)

Non-Hodgkin’s - NO B sx & presents IIB or LATER, spreads hematogenously - why it is much worse- can be anywhere

77
Q

Which form of lympohma has Pel-epstein fevers, and alcohol LN?

A

Pel- epstein fevers = CYCLICAL FEVERS

When they consume alcohol - non-tender LN become tender

78
Q

Which form of hodgkinds has burkitt’s lympoma and extra-nodal disease?

A

Burkitt’s = stary sky pattern

Extra-nodal dz b/c spreads hematogenously

79
Q

Primary hemostasis physiology

A
  1. Endothelial damage
  2. VWB factor comes out, acts like velcro that attaches to glycoproteins on platelets= ADHESION
  3. Plts get activated (release TXA2, ADP) activates other plt nearby
  4. Act of other plt = plt aggregation
  5. Finishes w/ fibrinogen mesh across plt = plt plug = END Of primary hemostasis
80
Q

Secondary hemostasis physiology

A

ABOUT BLOOD FACTORS = intrinsic & extrinsic pathway & common pathway (X, V, II, I firbrinogen) –> Ia (fibrin)

Plt keep fibrin around so that when it’s activated can form full fibrin clot

Don’t want clots forming everywhere so have a way to break down clots via d-dimers - controlled by plasmin from plasminogen (activated to plasmin via TPA, why we give TPA as a clot buster = active plasmin to break down clots)

MAIN POINT - PRIMARY HEMOSTASIS = PLT

SECONDARY HEMOSTASIS = CLOTTING FACTORS

81
Q

Someone comes in with bleeding - what to order?

What does primary hemostasis bleeding look like?

A

Want to categorize as plt bleeding…primary hemostasis (order CBC) vs secondary hemostasis (factor bleeding) ..order PT/aPTT

Plt bleeding - issues w/ primary hemostasis - SURFACE BLEEDING =
Gingival bleeding
Vaginal bleeding
Skin - petechiae

Clotting factor bleeding (issue w/ secondary hemostasis = DEEP BLEEDS:
Hemarthrosis
Hematoma (in muscle)

82
Q

Platelet bleeding is caused by?

A

Decreased plt NUMBER (thrombocytopenia) via:

  1. DESTRUCTION (DIC, TTP, ITP, HIT)
  2. PRODUCTION (bone marrow issue - MDS, CA, aplastic anemia), or
  3. SEQUESTRATION (cirrhosis - spleen sequesters)

Or NO PLT FUNCTION -
1. Iagrotenic via drugs (NSAIDs, ASA, clopidogrel)

  1. Uremia - causes plt dysfunction
  2. VON WILLEBRANDS DISEASE - TEST LOVES THIS (deficient in VWF & thereby factor VIII b/c VWF stabilizes factor VIII)
83
Q

Von willebrands disease

Patho
Dx
Tx

A

Patho:
Deficient in VWF - VWF also stabilizes factor VIII so you have a deficiency in factor VIII as well

Presentation:
Plt bleed w/ NORMAL COUNT & not be on an anti-plt drug

Dx:
VBF assay - will be low

Tx:
GIVE DDAVP to force it to express all of the VBF that they have
Also in crisis give Factor VIII!

Note: **May present w/ DEEP bleed b/c of factor VIII disease

84
Q

When PT/PTT are elevated what is the next thing you do?

Next thing to order?

A

MEANS there’s a DEEP bleed (you’ll already know this = hematoma, hemarthrosis)

Either from -clotting factor inhibitors OR from factor deficiency…how to tell?

Next thing to order = MIXING STUDY …

DOES NOT CORRECT w/ mixing study = 2/2 inhibitor

DOES CORRECT w/ mixing study = 2/2 CLOTTING FACTOR DEFICIENCY (warfarin therapy, vitamin K deficiency, DIC, VWF (b/c of factor VIII instability, and hemophilia A and B)

85
Q

Hemophilia A

Patho
CP
Tx = factor VIII concentrate

A

Patho = Factor VIII deficiency 2/2 X-linked recessive - MC bleeding d/o after VWD

CP = spontaneous hemarthrosis

Dx =
aPTT = prolonged (remember aPTT is intrinsic = factor VIII)

PT and bleeding time NORMAL

Tx = Refer to Heme/onc factor VIII concentrate

86
Q

Warfarin affects intrinsic or extrinsic coagulation pathway?

A

Extrinsic - remember OUTpatient’s - AKA Extrinsic = on warfarin –> get PT/INR

87
Q

aPTT measures intrinsic or extrinsic?

A

aPTT measures INTRINSIC

Remember IN-patient’s are on Heparin / factor VIII pathway

warfarin = outpatient = EXTRINSIC pathway

88
Q

Measure PT/INR Or aPTT for warfarin patients?

A

Measure PT/INR b/c warfarin affects extrinsic pathway

89
Q

Coagulopathy of liver disease?

PT/aPTT = ?
Response to vitamin k?

A

Liver is site of all factors except factor VIII so if coagulopathy 2/2 liver disease =

PT prolonged > aPTT (aPTT is pathway with factor VIII so it’s kind of okay)

NO RESPONSE TO VITAMIN K

If def in K+ b/c of alcoholic or deficient in vitamin K b/c no leafy greens then PT will NORMALIZE

90
Q

Coagulopathy of liver disease?

PT/aPTT = ?
Response to vitamin k?

A

Liver is site of all factors except factor VIII so if coagulopathy 2/2 liver disease =

PT prolonged > aPTT (aPTT is pathway with factor VIII so it’s kind of okay)

NO RESPONSE TO VITAMIN K

Will also have LOW

(If def in K+ b/c of alcoholic or deficient in vitamin K b/c no leafy greens then PT will NORMALIZE but in liver disease it’s so pooped out that giving more substrate = ZERO response)

91
Q

Common genetic causes of thrombosis

A

Factor V leiden (MC)

AT III deficiency

Protein C or S deficiency

Anti-phospholipid antibody syndrome (anti-cardiolipin antibodies)

92
Q

Labs of acute liver issue

A

Acute = marked elevation of ALT>AST

Elevated bilirubin

NORMAL INR/normal ALBUMIN

93
Q

Chronic liver issue labs

A

Minor elevation of ast/alt (no hepatocytes left to secrete even…)

Elevation of bilirubin

PROLONGED INR
HYPOALBUMINEMIA (this is what kills patients…need albumin for everything)

94
Q

1 cause of unconjugated hyperbilirubinemia?

A

HEMOLYSIS = a HEMATOLOGY PROBLEM, not a biliary tract problem!!!

Or rarely, an inherited disease (gilbert’s)

95
Q

Clinical presentation of a person with unconjugated hyperbilirubinemia? Most common cause?

A

CAUSE = HEMOLYSIS (unless it’s inherited then it’s Gilbert’s)

Presentation:
Cannot pee out the bilirubin b/c unconjugated is not water soluble so stool & urine are normal in color

Mildly jaundiced

*PANCE by subspecialty (22 decks)

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