Hem Exam 2 Flashcards

1
Q

How to manage bleeding

A

identify and manage bleeding source
give blood is Hgb <7 g/dL
reverse offending agent or hold dose

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2
Q

What is FFP (fresh frozen plasma)

A

reverses warfarin
frozen within 8 hours
will raise factor levels by 20%
can cause TACO (transfusion associated circulatory overload) 10-20 ml/kg/dose
lower INR 1.6

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3
Q

What is recombinant factor VIIa (novoseven, already activated factor)

A

reverse: LMWH, UFH, warfarin
for bleeding associated with hemophilia
(can use for intracranial hemorrhage and refractory bleeding after cardiac surgery)
Boxed warning: serious arterial and venous thrombotic events
activated extrinsic pathway

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4
Q

What is Prothrombin complex concentrate (PCC)

A

reverse: warfarin, off-label DOAC, DTIs
feiba: 2, 9, 10, 7
kcentra: 2, 9, 10, 7, C, S (IV with vit K)

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5
Q

Boxed warning for PCC and advantages

A

fatal and nonfatal arterial and venous thromboembolic events
lower, volume, low risk of transmission of infection, fast reversal
FACTOR 9

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6
Q

What is protamine

A

reveral: heparin, enoxaparin (hypersensitivity in fish)
-1 mg protamine neutralizes 100 units heparin (max dose 50 mg)
will not completely neutralize enoxaparin
BBW: hypotension, CV collapse, pulmonary vasoconstriction

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7
Q

Protamine doses (IV UFH)

A

stop drip, 1 mg per 100 units administered in last 3 hrs
max 50 mg
may repeat 0.5 mg for every 100 units in 15 minutes if bleeding or elevated aPTT/aXa
reduce dose by 50% if time (>2 hours) has elapsed since dose given

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8
Q

Protamine doses (SQ UFH)

A

only if pt has significant bleeding
1 mg per 100 units heparin, max 50
over 10 min and infuse the rest over 8-16 hours

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9
Q

What is Vit K

A

reversal: warfarin
IV or PO
give over 30 min, use PO mainly
0.5-10 mg
takes long time to work

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10
Q

What is andexanet alpha (andexXa)

A

reverse: apixaban, rivaroxaban, edoxaban
low dose: 400 mg IV then 4 mg/min for up to 2 hr
-apixaban 5, 10 or any dose given more than 8 hr ago
high dose: 800 mg IV then 8 mg/min for up to 2 hr
-if dose was given within last 8 hours or time unknown

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11
Q

What is idarucizumab (praxbind)

A

reverse: dabigatran
w/in minutes
5 g (2.5x2 vials), give first dose over 5-10 minutes, second dose given immediately after
no repeat doses

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12
Q

LMWH/fondaparinux TO IV heparin

A

start IV heparin without a bolus 1-2 hours before next dose is due

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13
Q

Warfarin TO heparin

A

IV heparin without bolus when INR is around 2

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14
Q

DOAC TO heparin

A

start IV without bolus when DOAC next dose is due

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15
Q

If on DOAC and need heparin when not urgent vs urgent

A

not: baseline aXa if high check q6h and start drip one level <0.7 units/mL
urgent: baseline aXa and aPTT, if aXa high, then titrate heparin based on aPTT, do not delay drip

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16
Q

LMWH/fondaparinux FROM IV heparin

A

stop heparin and start within 1 hour
-if aPTT/aXa subtherapeutic give at same time drip stopped
-if aPTT/aXa supra delay for longer (3-4 hours)

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17
Q

Warfarin FROM heparin

A

start warfarin and continue IV heparin until INR os therapeutic 1-2 times

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18
Q

DOAC FROM heparin

A

start DOAC when IV heparin is stopped
-if aPTT/aXa is supra consider delaying start time

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19
Q

DOAC to LMWH

A

begin LMWH at time next DOAC dose due

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20
Q

warfarin to LMWH

A

being LMWH once INR is around 2

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21
Q

DOAC from LMWH

A

start DOAC 2 hours prior to next dose of LMWH

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22
Q

warfarin from LMWH

A

start warfarin and continue LMWH until INR therapeutic 1-2 times

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23
Q

Warfarin to DOAC (target INR)

A

Rivaroxaban: <3
Edoxaban: <2.5
Apixaban: <2
Dabigatran: <2

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24
Q

Apixaban and RIvaroxaban to Warfarin

A

stop DOAC then start warfarin on same day and bridge until INR is therapeutic 1-2 times

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25
Q

Edoxaban to warfarin

A

60 mg qd, reduce to 30 mg and begin warfarin at same time
30 mg qd, reduce to 15 mg and begin warfarin at same time
-disc edoxaban once INR >2 1-2 times (get INR right before edoxaban dose

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26
Q

Dabigatran to warfarin

A

stop DOAC then start warfarin on same day, bridge until INE therapeutic 1-2 times
CrCl >50 ml/min: start warfarin 3 days before stopping DOAC
CrCl 30-50: start warfarin 2 days before stopping
CrCl: 15-30: start warfarin 1 say before stopping

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27
Q

Argatroban to Warfarin

A

begin warfarin and stop argatroban once INR >4 (and 5 days minimum overlap if indicated)
-repeat INR in 4-6 hours, if INR is still therapeutic then no argatroban
-INR below goal, resume argatroban drip
Can hold for 2-3 hours and check INR

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28
Q

Bival to Warfarin

A

begin warfarin and stop bival once INR >2 (and 5 days min overlap if indicated)
repeat INR in 2 hours, if INR therapeutic no drip
if INR below goal, resume drip

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29
Q

DOAC to DOAC

A

give are next scheduled dose

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30
Q

Low thrombotic risk

A

C2V <4, no stoke hx
VTE >12 months
MHV: AV bioprosthetic

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31
Q

Moderate thrombotic risk

A

C2V: 5-6 or stroke >3 months ago
VTE past 3-12 months
active cancer
MHV: AV bioprostheitc, one or more risk factors

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32
Q

High thrombotic risk

A

C2V >7, stroke <3 months
VTE <3 months
MHV: any MV prosthesis

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33
Q

What does the CHADS2-VASC score indicate

A

stroke rate %

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34
Q

What is the HAS_BLED score for

A

a fib
major bleeding risk

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35
Q

Minimal bleed risk assessment

A

minor dental
cataract/glaucoma procedure
diagnostic GI procedure
superficial incisions

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36
Q

Low bleed risk assessment

A

colonoscopy
pacemaker implantation
ICD placement
AV nodal ablations
percutaneous coronary intervention

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37
Q

High bleed risk assessment

A

surgery needed neuraxial anesthesia
major intracainal surgery, thoracic, CABG, arthroplasty, bowel resection

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38
Q

Dabigatran (CrCl >50 ml/min) and rivaroxaban, apixaban, edoxaban high vs low bleed risk interruption

A

high: 2 days before until 1 day after
low: 1 day before and day of procedure

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39
Q

Dabigatran (CrCl <50 ml/min) high vs low bleed risk interruption

A

high: 4 days before until 1 day after
low: 3 days before until day of procedure

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40
Q

Low thrombotic risk no pt bleed factors: low, high, uncertain procedure bleed risk

A

minimal/low: do not interrupt
inter/high: likely interrupt/do not bridge
uncertain: likely interrupt/do not bridge

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41
Q

Moderate thrombotic risk no pt bleed factors: low, high, uncertain procedure bleed risk

A

minimal/low: do not interrupt
inter/high: likely interrupt, likely dont bridge (unless recent stroke, TIA, SE)
uncertain: likely interrupt, likely dont bridge (unless recent stroke, TIA, SE)

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42
Q

high thrombotic risk no pt bleed factors: low, high, uncertain procedure bleed risk

A

minimal/low: do not interrupt
inter/high: interrupt and bridge
uncertain: interrupt and bridge

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43
Q

low thrombotic risk pt bleed risk factors, all bleed risk factors

A

likely interrupt/do not bridge

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44
Q

moderate thrombotic risk pt bleed risk factors, all bleed risk factors

A

likely interrupt/do not bridge

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45
Q

high thrombotic risk pt bleed risk factors, all bleed risk factors

A

likely interrupt, likely bridge (unless major bleed or ICH <3 months)

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46
Q

Difference between HIT 1 and HIT 2

A

HIT 1: non-immune mediated, day 1, mild rxn
HIT 2: immune-related, heparin induced (90d), delayed onset 5-14d, life-threatening

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47
Q

Risk factors for HIT

A

post-surgery, trauma
CV > ortho surgery
UFH > LMWH > fondaparinux
duration
female > male
bovine > porcine UFH

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48
Q

Pathophysiology of HIT

A

heparin + PF4
heparin-PF4 complex
IgG antibody production
IgG antibody + heparin/PF4 immunocomplex
binding of complex to platelets
platelet activation and aggregation

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49
Q

Mechanism of thrombosis vs thrombocytopenia

A

thrombosis: arterial or venous, activate platelets and injury endothelial cells
thrombocytopenia: removal of IgG coated platelets by macrophages, consumption of platelets at sites of thrombosis

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50
Q

How to calculate 4T:

A

Thrombocytopenia: 2 (decrease PLT 50%), 1 (30-50)
Timing: 2 (5-10d, or 1 d with heparin), 1 (fall not clear 5-10), 0 (PLT fall <4 d without exposure)
Thrombosis: 2 (new), 1 (progressive)
oTher causes: 2 (none), 1 (possible), 0 (definite)

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51
Q

Immunoassays: PF4 EIA serologic test

A

high sensitivity but low specificity
optical density scoring
test for HIT

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52
Q

Functional Assay: serotonin release assay (SRA)

A

gold standard for HIT
detects platelet-activating properties

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53
Q

What is the chronic phase of HIT

A

subacute HIT A: period of PLT recovery, functional assay and immunoassay positive
subacute HIT B: functional assay becomes negative, immunoassay remains positive

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54
Q

What to do if HIT suspected then 4T score is high vs low

A

high: disc heparin -> immunoassay -> negative HIT unlikely, positive treat tx or ppx -> if functional assay positive start HIT management
low: HIT unlikely, no lab testing, continue heparin if needed

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55
Q

Acute Phase HIT treatment

A

Argatroban (critically ill, avoid in hepatic dysfunction)
Bival (critically ill, avoid in renal)
DOAC (stable, avoid in complicated, threatening emboli)
Warfarin (NOOOOO)

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56
Q

Chronic Phase HIT treatment

A

DOAC over warfarin
3-6 months care if thrombus
cont tx until PLT recovery
avoid tx in >3 months isolated HIT
Warfarin is PLT >150, Edoxaban/dabigatran with 5 d overlap

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57
Q

Monitoring for HIT

A

renal/hepatic
CBC
PT/INR (warfarin)
aPTT (agatroban/bival)
S/S of thrombosis
S/S of bleeding

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58
Q

Goals of HIT therapy

A

halt platelet activation as rapidly as possible
provide therapeutic-dose anticoag with non-heparin
prevent thrombosis
prevent mortality
avoid heparin products in future

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59
Q

hemoglobin criteria for men and women

A

men: <13
women: <12
anemia is a disorder resulting in reduced oxygen-carrying capacity of the blood

60
Q

Stimulation of erthropoiesis

A

feedback loop:
decrease tissue oxygen concentration
hormone EPO gets produced in kidneys
EPO gets released into plasma
stimulate production of RBC in bone marrow
stimulated maturation of RBCs
(RBCs maruration is 1 wk)

61
Q

What does transferrin do

A

delivers iron to bone marrow to be incorporated into the Hb
extra iron stored as ferritin

62
Q

Red blood cell destruction

A

spleen breaks down RBC
average life span 120 days

63
Q

Clinical presentation symptoms of anemia

A

fatigue, headache, yellow skin, irregular heartbeat, chest pain, cold hands, dizziness, leg cramps, insomnia
(due to lack of oxygen to tissues)

64
Q

Clinical presentation signs of anemia

A

pallor (iron deficiency)
abnormal gait (B12 deficiency)
Decreased mental acuity
tachycardia

65
Q

Common causes of anemia

A

deficiency of iron, B12, folic acid
cancer
chronic diseases: kidney, HF, liver, inflammation
blood loss: GI bleed, surgery, injury, menstruation

66
Q

CBC components: hemoglobin, hematocrit, RBC

A

hemoglobin: amount of hemoglobin per volume of whole blood
hematocrit: volume of RBCs in a unit volume of whole blood (%)
RBC: total count of RBCs per unit of blood

67
Q

mean cell volume, meant cell hemoglobin, mean cell hemoglobin concentration

A

MCV: average volume of RBCs
MCH: average amount of Hb in a RBC
MCHC: concentration of Hb per volume of cells

68
Q

Microcytic, normocytic, macrocytic

A

Microcytic: MCV < 80 (iron deficiency, chronic disease)
Normocytic: 80-100 (acute blood loss, bone marrow failure
Macrocytic: >100 (alcoholism, liver, hypothyroidism, folic acid deficiency, Vit B12 deficiency

69
Q

Total reticulocyte count, red blood cell distribution width (RDW)

A

Total reticulocyte count: measures of new RBC production (immature cells), low = impaired production, high = acute blood loss
Red blood cell distribution width (RDW): distribution of RBC size, higher = more variable sizes, harmful for anemias

70
Q

Iron studies: serum iron, total iron binding capacity, transferrin saturation, serum ferritin

A

serum iron: concentration of iron bound to transferrin (diurnal)
TIBC: indirect measurement of iron-binding capacity of serum transferrin (high=bad)
TSAT: ratio of serum iron to TIBC (low=bad)
Serum Ferritin: total iron storage (low=iron def)
-general population ferritin <30 (greater than or equal to)

71
Q

Folic acid, B12, homocysteine, methylmalonic acid (MMA)

A

folic acid: <4 decreased serum indicates deficiency
B12: <200 is deficiency
homocysteine: increased levels could mean either
MMA: increased levels mean B12

72
Q

Low MCV (<80) meaning if serum ferritin is normal/high or low

A

normal/high: low TIBC = anemia of chronic disease, high = further work up
low (<30): iron deficiency anemia

73
Q

Normal MCV (80-100) meaning for low or high reticulocyte count

A

low: check WBC/platelets -> low = bone marrow failure, high=infection

74
Q

High MCV (>100) meaning if B12 level is normal and folate is decreased

A

normal B12, normal folate: hepatic, hypothyroidism, drug anemia, reticulocytosis
decreased B12, normal folate: Vit B12 deficiency

75
Q

What regulates intestinal iron absorption

A

hepcidin (normal iron content: 3-4g)

76
Q

Increased risk for anemia

A

children <2
adolescents
pregnant females (30 mg/d)
>65 yo

77
Q

Specific symptoms with IDA

A

glossal pain
smooth tongue
pica (compulsive eating of nonfood items)
pagophagia (compulsive ice eating)

78
Q

IDA lab finding

A

low serum iron
low ferritin levels (<30)
high TIBC**
Hg, Hct, RBC normal at first then drop

79
Q

Pharm Tx for IDA

A

ferrous gluconate: 38 mg elemental iron
ferrous sulfate: 65 mg (syrup, elixir)
ferrous fumarate: 106 mg
PIC (come in liquid)

80
Q

Adverse effects of oral iron supplements

A

dark colored feces
ab pain
indigestion
constipation
n/v

81
Q

Monitoring parameters of oral iron supplements

A

CBC (Hg/Hct)
RBC indices
Iron
(peak 4 weeks, repeat lab 1-3 months, cont supply for 3-6 months after labs normalize

82
Q

Clinical pearls of iron supplements

A

start low and slow
food decrease absorption (take with acidic drinks)
1 hr before or 2 hours after food
fatal to children/pets
add stool softener to prevent constipation

83
Q

What drugs decrease Iron supplements

A

antacids
tetracyclines
H2RA
PPI
cholestyramine

84
Q

What drugs does iron supplements decrease their absorption

A

levodopa
methyldopa
levothyroxine
penicillin
fluoroquinolones
tetracyclines
mycophenolate

85
Q

Treatment failure for oral iron supplements

A

poor patient adherence
poor absorption
incorrect diagnosis
continued bleeding
concurrent inflammatory process limiting therapeutic response

86
Q

When to use IV iron over oral

A

not tolerating orals
malabsorption
non-adherence
significant blood loss

87
Q

Parental Iron Products

A

None: Iron dextran
CKD: Ferric Carboxymaltos
CKD and HD:
Sodium Ferric gluconate
Iron Sucrose
ferumoxytol

88
Q

Adverse effects of IV iron products

A

infection reactions
arthralgia/myalgias
hypotension
tachycardia
chest pain
peripheral edema
pruritis

89
Q

Monitoring parameters for IV iron products

A

REACTIONS (during/post infection)
CBC (Hb/Hct)
RBC indices
Iron studies
1-3 months

90
Q

Etiology of B12 deficiency

A

inadequate intake
Malabsorption
inadequate utilization (low gastric acid production, metformin, H2RA, PPI >2 years of use)

91
Q

B12 deficiency symptoms

A

bilateral paraesthesia
deficits in proprioception and vibration
ataxia
dementia-like symptoms
psychosis
vision loss

92
Q

B12 deficiency lab findings

A

Macrocytic anemia >100
leukopenia/thrombocytopenia
low reticulocyte count
low serum B12 <200
Low Hct
elevated MMA/homocysteine

93
Q

B12 treatment

A

cereal, fish, liver, milk, clams, yogurt
oral: 1000-2000 mcg qd
SQ/IM: 1000 mcg inj d x7d then weekly x1 month then monthly
ALWAYS give if presenting neurologic
Intranasal: 500 mcg (1 spray) qw for maintenance

94
Q

What is the active form of folic acid

A

tetrahydrofolate
works with Vit B12 as a cofactor

95
Q

Folic Acid etiology: inadequate intake and increased folate requirements

A

inadequate intake: elderly, teens, alcoholics
increased folate requirements: pregnancy, infancy, malignancy, inflammatory disease, burn pt, dialysis

96
Q

Folic acid medications that decreased absorption

A

methotrexate
trimethoprim
phenytoin
phenobarbital

97
Q

Folic Acid deficiency lab findings

A

normal B12 and normal MMA (B12 deficiency)
Serum folate: <3
RBC folate: <150
homocysteine elevated

98
Q

Treatment for folic acid

A

oral: 0.4, 0.8, 1 mg qd
IV: rare
beef liver, lentils, leafy veggies, cereals, OJ, rice

99
Q

Adverse effects of folic acid

A

IV: pruritus, skin rash, flushing, bronchospasms

100
Q

Efficacy for folic acid

A

cont at least 4 months to correct folate deficient RBC in circulation
may need long-term in chronic conditions
repeat CBC, reticulocyte count, folic acid level in 4 months

101
Q

Anemia of Inflammation (AI)

A

ACD: anemia of chronic disease, develop over mon to yr
ACI: anemia of critical illness, rapid onset to tissue damage and acute inflammation

102
Q

Etiology of AI

A

multifactorial, usually a diagnosis of exclusion
infection, inflammation, tissue injury, proinflam cytokine
shorter RBC lifespan
disturbance of iron hemostasis
aspirin therapy, NSAID, anticoag, corticosteriods

103
Q

Examples of ACD

A

chronic infections: TB, HIV, Osteo, endocarditis
chronic inflammation: RA, SLE, IBD, gout
Malignancies: carcinoma, lymphoma, leukemia, multiple myeloma
Other: alcohol liver disease, HF, COPD

104
Q

Underlying causes in ACI

A

sepsis and increased metabolic demands
frequent blood sampling
surgical blood loss
immune-mediated functional iron deficiency
decreased production of EPO
reduced RBC life span
nutritional deficits

105
Q

AI lab findings

A

no definitive test to confirm
ACD mild-moderate (Hb >8-9.5)
can coexist with other anemias
normocytic

106
Q

IDA vs AI lab findings: Iron, TIBC/transferrin, TSAT, ferritin

A

AI: increased ferritin, others decreased/normal
IDA: increased transferrin, others all decreased

107
Q

Treatment for AI (iron supplementation only effective if iron deficiency is also present)

A

Erythropoietin Stimulating Agents (ESAs)
No HgB >10
for CKD, HIV, malignancy
products are not interchangeable
reserved for severe anemia (HgB <7-8)

108
Q

Epoetin Alfa (epogen, procrit) MOA

A

glycoprotein that stims RBC production
stims division and differentiation of progenitors in bone marrow
endogenous EPO production feedback loop regulation

109
Q

Epoetin Alfa (epogen, procrit) indications and contraindications

A

anemia in cancer pt due to chemo, CKD, HIV
not: serious allergic rxn to drug, uncontrolled HTN, pure red cell aplasia (PRCA)

110
Q

Epoetin alfa adverse drug reactions

A

hypersensitivity, headache, pruritus, n/v, injection site pain, fever, arthralgias
increased risk for seizures, pure red cell aplasia

111
Q

Epoetin alfa BBW

A

CV events: risk of MI, stroke, VTE, HTN
increased risk of death, CV event, stroke when Hb >11
increased tumor progression in cancer

112
Q

Epoetin alfa dosing

A

50-150 u/kg sq 3 times weekly
if Hb increase by >1 in 2 wks or >2 on 4 wks, decrease dose by 25-50%
if no increase by 1 after 4 wks increase dose by 25%
Hb target: 10-11.5
disc therapy if no response 4-12 wks

113
Q

Darbepoetin Alfa (aranesp): MOA, tx, CI

A

stim division of erythroid progenitors in bone marrow
longer half life
for anemia in cancer and CKD
CI in serious allergic rxn, PRCA

114
Q

Darbepoetin alfa ADE and BBW

A

hypersensitivity, edema, ab pain, dyspnea, cough, increased risk of seizures, PRCA
CV events (same as epoetin alfa)

115
Q

Darbepoetin alfa dosing

A

0.45-2.25 qw, 0.75 q2w, 500 mcg q3w
>1 inc in 2 w or >2 inc in 4 w, dec dose by 25-50%
Hb no inc by >1 in 4 wk inc dose by 25%
HB target (10-11.5)
no response in 4-12 wk disc

116
Q

ESA risk/benefits

A

risk: increase thrombotic events, decrease survival, increased time to tumor progression
benefit: transfusion avoidance, gradual improvement s/sx

117
Q

RBC transfusion risk/benefit

A

risk: increase thrombotic events, decrease survival, transfusion rxn, TACO, virus transmission, bacterial contamination, iron/vol overload
benefit: rapid increased Hb/Hct, improved s/sx

118
Q

Goals of therapy

A

return hematologic parameter to normal
QOL
prevent long-term complications
treat underlying disorder
correct reversible causes of anemia
B12: prevent/resolve neurologic sx

119
Q

Sickle cell syndromes

A

SCT: sickle cell trait
HbAS
SCD: sickle cell disease
HbSS (sickle cell anemia)

120
Q

What is the point mutation in HbS polymerization

A

glutamic acid to valine
-alters RBC shape, density, membrane, adhesion, ability to deform into sickle shapes

121
Q

Pathophysiology of sickle cell anemia

A

HbS sickles leading to RBC hemolysis
they interact with neutrophils and PLTs leading to vaso-occlusion
HbF (fetal) is immune to sickling
occurs when RBCs are deoxygenated

122
Q

Symptoms of SCA

A

fever
arthralgia
ab pain
weakness and fatigue
weight loss
hematuria
pain and swelling in hands and feet

123
Q

Signs of SCA

A

chronic anemia/pallor
anorexia
hematuria
delayed growth in children
enlargement of liver, spleen, heart
increased reticulocytes, lactate dehydrogenase, PLT count, WBC count
normal MCV
sickle RBSs on peripheral smear
signs of VTE

124
Q

Diagnosis of SCA

A

heel stick test preformed w/in 24-48 hrs of birth
positive screening should be confirmed by 2nd test before 2 months of age

125
Q

Acute SCA complications

A

acute pain crisis
acute chest syndrome
stroke
venous thromboembolism
cholecystitis
priapism
infection
splenic sequestration
vaso-occlusive crisis

126
Q

Chronic SCA complications

A

chronic pain
iron overload
osteonecrosis
retinopathy
nephropathy
HF
pulmonary HTN
leg ulcers

127
Q

Hydroxyurea (gold standard for SCA) reduces the symptoms of what

A

frequency of acute pain crises
episodes of acute chest syndrome
blood transfusions
hospital stays
prevents/slow organ damage

128
Q

Hydroxyurea MOA

A

inhibits DNA synthesis

129
Q

Hydroxyurea indication

A

> 9 months old with SCD
3 or more moderate-to-severe pain crises per year
h/o severe or recuttent acute chest syndrome

130
Q

Hydroxyurea boxed warnings and ADE

A

BBW: myelosuppression, malignancy
ADE: increased lft, uric acid, BUN, SCr, mouth ulcers, N/V/D, alopecia, hyperpigmentation or atrophy, low sperm count

131
Q

Hydroxyurea dosing

A

15 mg/kg/d adults
20 mg/kg/d children
maintenance: titrated based on CBC, 20-35 mg/kg/d
max: 2500 mg qd
(comes in 50 mg increments)

132
Q

Hydroxyurea monitoring

A

CBC with diff
HbF
uric acid
renal function/LFTs
baseline pregnancy test

133
Q

Hydroxyurea treatment goals

A

less pain and ACS episodes
increase HgF
increase HgB
ANC 2000-4000

134
Q

Hydroxyurea toxicity

A

myelosuppresion
-ANC <2000
-PLT <80,000
-Reticulocyte <80,000 if Hgb <9
-HgB <5 or more than 20% at baseline
Increase SCr 50% above baseline
Increase ALT 100% above baseline

135
Q

Hydroxyurea titration

A

clinical response 3-6 months
CBC with diff q2-4wk
increase 5 mg/kg/d q8-12w
if toxicity then hold
restart 2.5-5 mg/kg/d
CBC q2-3 months once stable dose achieved

136
Q

L-glutamine MOA

A

precursor for nicotinamide adenine dinucleotide (NAD+) synthesis

137
Q

L-glutamine indication, monitoring, ADE

A

5 years and older
monitor: renal and hepatic function
ADE: constipation, ab pain, nausea

138
Q

Crizanlizumab MOA

A

binds and inhibits P-selectin
P-selectin helps sickle cells to adhere to vessels

139
Q

Crizanlizumab indication, monitoring, and ADE

A

16 years and older
monitor: signs and symptoms of infusion-related rxns
infusion-related rxn, headache, arthraliga, diarrhea, pruritus, vomiting, chest pain

140
Q

Non-pharm SCA

A

blood transfusions
bone marrow transplant
gene therapy
hematopoietic stem cell transplant

141
Q

Acute SCA fever and infection

A

functional asplenia w/in 1st year of life
increased sick for serious infections
Strep pneumoniae, n. meningitis, h. influenzae
fever = bad = sepsis (>38.5C/101.3F)

142
Q

When to use penicillin in SCA

A

all infants screened positive at birth
continue until 5 yo unless invasive pneumococcal infection or splenectomy

143
Q

SCA pain crises

A

most common complication
leading cause ED visits
assess pain and admin med w/in 1 hr of presentation

144
Q

SCA pain crises management

A

hydration: 1-1.5x maintenance fluid requirement
Mild: NSAID/APAP (codeine or hydrocodone if worse)
Severe: morphine, methadone, ketamine

145
Q

Acute chest syndrome

A

new pulmonary infiltrate associated with fever/respiratory symptoms
pulmonary vascular occlusion and infection
Abx: macrolide/quinolone

146
Q

Acute chest syndrome care

A

avoid analgesic-induced hypoventilation
steroids for inflammation
oxygen for supportive care

147
Q

Goals of SCA therapy

A

reduce hospital
reduce acute and chronic complications
improve qol
prevent death