Hematology

Question 1

what are the 3 layers of blood vessels? (I, M, A)

Answer 1

intima, media, adventitia

Question 2

Vessels:

1. plts adhere to what? (IVW)
2. what 2 mediators cause the vessel contract after injury? (T, A)
3. what mobilizes during the adhesion phase from endothelial cells? (V)
4. what attaches to vWF and attracts plts to bind to endothelial lining? (G)
5. what do vWF make plts, which allows them to adhere to the injury site? (S)

Answer 2

1. injured vessel wall
2. thromboxane, ADP
3. vWF
4. glycoprotein Ib
5. sticky

Question 3

Coagulation factors:

1. what 4 factors are vitamin K dependent?
2. what factor is fibrinogen?
3. what factor is prothrombin?
4. what factor mediates adhesion?
5. where are most of these synthesized?

Answer 3

1. 2, 7, 9, 10
2. 1
3. 2
4. vWF

Question 4

what are the factors in order for these coagulation cascades:

1. extrinsic (4)
2. intrinsic (7)

Answer 4

1. 7, 10, 2, 1

2. 12, 11, 9, 8, 10, 2, 1

Question 5

Meds that influence coagulation:

1. what are the 4 anticoagulants? (H, L, CD, TI)
2. what med is a procoagulant?
3. what 3 meds are antiplatelet? (P, N, T)
4. what 2 meds are antifibrinolytic? (T, A)

Answer 5

1. heparin, LMWH, coumadin derivatives, thrombin inhibitors
2. vitamin K
3. persantine, NSAIDs, thienopyridine
4. TXA, amicar

Question 6

coagulation tests:

1. bleeding time (minute, range)
2. plts (per mm cubed, range)
3. thrombin T (seconds, range)
4. PT average (seconds, range)
5. aPTT average (seconds, range)
6. ACT (seconds, range)
7. FDP < what mcg/mL?
8. D-dimer less than what mg/mL?

Answer 6

1. 3-7 min
2. 150-350K
3. 8-12 sec
4. 12-14 sec
5. 25-32 sec
6. 80-150 sec
7. < 10 mcg/mL
8. < 500 mg/mL

Question 7

what are the 3 reasons for blood transfusions? (RV, RCF, IOCC)

Answer 7

1. replace volume
2. replace coagulation factors
3. improve oxygen carrying capacity

Question 8

does oxygen-carrying capacity of RBCs increase or decrease with length of storage?

Answer 8

decrease

Question 9

does oxygen carrying capacity improve when 2,3 DPH is regenerated once transfused?

Answer 9

yes

Question 10

plt infusion guide:

1. recommended dose is 1 plateletpheresis pack per how many kg of body weight?
2. how is the normal lifespan of donate plts? (range, days)
3. 1 pack of plts increases the plt count by what range?
4. plt count less than what is associated with increased intraoperative blood loss?
5. stored at room temperature for what day range?

Answer 10

1. 10 kg
2. 4-5 days
3. 5,000-10,000
4. 50,000
5. 4-5 days

Question 11

what blood product as the greatest risk of bacterial transmission?

Answer 11

plts

Question 12

FFP guide:

1. contains all of what? (CF&NCI)
2. average mL
3. do they need to be ABO compatible?
4. is it used for volume replacement?
5. is FFP indicated when PT or INR and aPTT are normal?

Answer 12

1. clotting factors and natural clotting inhibitors
2. 200 mL
3. yes
4. no
5. no

Question 13

reasons for FFP infusion:

1. urgent reversal of what med?
2. correction of what, when specific concentration is not available? (KFD)
3. correct excessive what with INR >2 in absence of heparin? (MB)
4. correction of microvascular bleeding d/t coag factor deficiency in pts transfused with greater than 1 what? (BV)

Answer 13

1. warfarin
2. known factor deficient
3. microvascular bleeding
4. blood volume

Question 14

Cryo infusion guide:

1. contains what 2 things and what 2 factors? (F, F)
2. used when fibrinogen is less than what mg/dL range and pt is bleeding?
3. used as an adjunct during what when fibrinogen can’t be quickly checked? (MT)
4. what are the 2 other reasons to give? (F, CFD)

Answer 14

1. fibrinogen, fibronectin, factor 8, factor 13
2. 80-100 mg/dL
3. massive transfusions
4. fibrinolysis, congenital fibrinogen deficiencies

Question 15

what are 4 blood transfusion strategies? (PAD, ANH, BCS, RF8)

Answer 15

1. preop autologous donation
2. acute normovolemic hemodilution
3. blood cell salvage
4. recombinant factor 8

Question 16

Hgb less than what triggers transfusion for hi risk pts?

Answer 16

< 7

Question 17

porphyrias:

1. inborn metabolic error secondary to what deficiency in the heme synthesis pathway? (E)
2. this deficient leads to an overproduction of what? (P)
3. porphyrin is needed for what 2 things with O2? (T, S)
4. defective enzyme leads to an accumulation of preceding intermediate form of what? (P)
5. what porphyria are we most worried about?
6. we are only worried about this form of porphyria because its the only form that can result in what type of reactions with certain drugs? (LT)

Answer 17

1. enzyme
2. porphyrin
3. transport, storage
4. porphyrin
5. acute intermittent porphyria
6. life-threatening

Question 18

Classes of porphyria:

1. what are the 2 types? (H, E)
2. class depends on the primary site of what or site of what of precursors or porphyrins? (O, A)

Answer 18

1. hepatic, erythropoietic

2. overproduction, accumulation

Question 19

acute porphyria:

1. inherited autosomal dominant or recessive?
2. attacks are more frequently in men or women in what 2 decades of life?
3. acute attacks are precipitated by events that decrease what concentration and increase what enzymes activity? (H, AS)
4. the above scenario is a stimulation of the production of what? (P)
5. what are the most important trigger for acute porphyria? (EID)
6. what are 6 other reasons that can precipitate an acute attack? (HF, M, F, D, S, I)
7. this disease is associated with what 3 things in pregnancy? (H, SA, LBW)

Answer 19

1. dominant
2. women, 3rd and 4th decade
3. heme, ALA synthetase
4. porphyrinogens
5. enzyme inducing drugs
6. hormone fluctuation, menstruation, fasting, dehydration, stress, infection
7. HTN, spontaneous abortion, low birth weight

Question 20

acute porphyria:

1. can pts with known risk for porphyria but previously asymptomatic experience their first symptoms of porphyria in response to triggering drugs given during the perioperative period?
2. what are 5 S/S? (SAP, AI, ED, NS, SMW, S)
3. CNS manifestations can be seen with what 3 things? (UMNL, CNP, C/BGA)

Answer 20

1. yes
2. severe abd pain, ANS instability, electrolyte disturbances, neuropsychiatric symptoms, skeletal muscle weakness, seizures
3. upper motor neuron lesions, cranial nerve palsies, cerebellar/basal ganglia abnormalities

Question 21

acute porphyria triggering drugs:

1. they induce what enzyme activity or interfere with what feedback control at final common pathway of heme synthesis? (AS, N)
2. allyl groups on what drug type and some structures of what other drug type are known to produce porphyria? (B, S)

Answer 21

1. ALA synthetase, negative

2. barbiturates, steroid

Question 22

acute porphyria:

1. avoid what 5 meds? (T, E, M, P, T)
2. probably avoid what 1 med? (N)

Answer 22

1. thiopental, etomidate, methohexital, pentazocine, thiamylal
2. nifedipine

Question 23

anesthesia and porphyria:

1. are pts with active porphyria or acute porphyric crisis at an increased risk?
2. is exposure to several enzyme-inducing drugs more dangerous than any one drug?

Answer 23

1. yes

2. yes

Question 24

porphyria and regional blocks:

1. what should be performed before the block? (NE)
2. ANS blockade secondary to regional block could unmask what instability especially in pts with what 2 things? (C; AN, H)
3. is there evidence that LA-induced acute porphyria attack or neurologic damage?
4. can a regional block be safely performed in parturients with acute intermittent porphyria?
5. not a good choice in pts with attack of acute intermittent porphyria d/t concerns with what 3 things? (HI, MC, N)
6. a neuro exam is performed before the block to decrease the likelihood of worsening what? (PEN)

Answer 24

1. neuro exam
2. C/V instability; autonomic neuropathy, hypovolemia
3. no
4. yes
5. hemodynamic instability, mental confusion, neuropathy
6. pre-existing neuropathy

Question 25

Porphyria and general anesthesia: 1. total dose and length of exposure to anesthetic drugs may influence the risk of triggering what? (PC) 2. is propofol safe for induction? 3. are all barbiturates unsafe, even if porphyria is in quiescent phase? 4. in these pts, consider frequent presence of what and possible what lability? (AD, BP)

Answer 25

1. porphyric crisis 2. yes 3. yes 4. autonomic dysfunction, blood pressure

Question 26

Cardiopulmonary bypass with porphyria: 1. is there an increased risk of porphyric crises in pts undergoing CPB? 2. what are the 5 theoretical risks of porphyric crisis as it relates to CPB? (H, PIH, BL, IHD, LNOD)

Answer 26

1. no | 2. hypothermia, pump-induced hemolysis, blood loss, increased heme demand, large number of drugs

Question 27

treatment of porphyric crisis: 1. remove any known what? (TF) 2. ensure adequate what and load with what? (H, C) 3. sedate with what drug? (P) 4. pain treated with what med type? (O) 5. what med type for increased HR and BP? 6. what med type to treat seizures? (B) 7. what 3 heme products can be given if no response to conservative therapy? (H, HA, HA) 8. N&V treat with what type of antiemetics? (C) 9. what electrolyte can be used to treat this?

Answer 27

1. triggering factors 2. hydration, carbohydrates 3. phenothiazine 4. opioids 5. beta blockers 6. benzos 7. hematin, heme albumin, heme arginine 8. conventional 9. magnesium

Question 28

acute intermittent porphyria: 1. is it the most likely to be life-threatening? 2. what is the defective enzyme? (PD) 3. what chromosome number? 4. affects what 2 nervous systems? 5. produces the most serious symptoms including what 2 things? (H, RD)

Answer 28

1. yes 2. porphobilinogen deaminase 3. 11 4. central and peripheral nervous system 5. HTN, renal dysfunction

Question 29

Plt disorders: 1. what fraction is sequestered in the spleen at any given time? 2. what is the plt life day range? 3. what number plt range must be produced daily to maintain a steady state?

Answer 29

1. 1/3 2. 9-10 days 3. 15,000-45,000

Question 30

what are 3 reasons that plt transfusion is needed? (LTH, BIACS, ES)

Answer 30

1. life-threatening hemorrhage 2. bleeding into a closed space (cranium) 3. emergency surgery

Question 31

long-term management of thrombocytopenia is therapy to improve what and to decrease what? (PP, PD)

Answer 31

1. plt production | 2. plt destruction

Question 32

Plt count needs to be greater than what for these surgeries: 1. minor surgery (range) 2. major surgery 3. neurosurgery

Answer 32

1. 20-30,000 2. 50,000 3. 100,000

Question 33

recheck plts how many hours after transfusion?

Answer 33

1 hour

Question 34

one unit of single-donor apheresis plts is equivalent to a random-donor pool of what range of units?

Answer 34

4-8 units

Question 35

it pts becomes alloimmunized to random-donor plts, blood bank can provide what type of plts? (H-M S-D)

Answer 35

HLA-matched single-donor

Question 36

do random and single donor plts need to be ABO compatible?

Answer 36

no

Question 37

women of childbearing age should receive Rh positive or negative plts?

Answer 37

Rh negative

Question 38

severe thrombocytopenia: 1. considered if plt is less than what? 2. petechial rash of what 2 places is consistent with thrombocytopenia? (S, MM) 3. petechial rash most pronounced in lower extremities d/t increased what pressure? (H) 4. thrombocytopenia differential diagnosis is based on what 3 things? (PP, PD, CD)

Answer 38

1. 15,000 2. skin, mucus membrane 3. hydrostatic pressure 4. plt production, plt destruction, circulation distribution

Question 39

platelet disorders: 1. what are the names of these 5 congenital plt disorders? (ADT, TARS, WAS, MHA, FA) 2. what are the 3 nonimmune plt destruction disorders? (TTP, HUS, HELLPS) 3. what are the 2 autoimmune platelet destruction disorders? (ITP, HIT) 4. what is the 1 qualitative plt disorder? (VWD)

Answer 39

1. autosomal dominant thrombocytopenia, thrombocytopenia-absent radius syndrome, wiskott-aldrich syndrome, may-hegglin anemia, fanconi's anemia 2. thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, HELLP syndrome (hemolysis, elevated liver enzymes, low plt count) 3. idiopathic thrombocytopenic purpura, heparin induced thrombocytopenia 4. von willebrand disease 5.

Question 40

acquired plt disorders: 1. results from damage where? (BM) 2. all aspects of normal hematopoiesis can be depressed to the point of bone marrow what? (A) 3. decreased marrow megakaryocyte mass can be due to what 5 things? (R, C, T, A, VH) 4. ineffective thrombopoiesis d/t alcoholism can because of deficiencies in what 2 things? (B, F) 5. if thrombopoiesis is caused by deficiency of the above 2 things, what therapy can be given? (VT) 6. after given this therapy, plts should be normal in how many days? (one word, F)

Answer 40

1. bone marrow 2. aplasia 3. radiation, chemo, toxins, alcohol, viral hepatitis 4. B12, folate 5. vitamin therapy 6. few days

Question 41

Nonimmune Platelet Destruction Disorders: 1. in DIC, the entire what is activated? (CP) 2. in DIC, there is severe thrombocytopenia and prolonged coagulation leading to a sharp increase in what? (B) 3. what are 5 causes of plt DIC? (VI, B, M, HDC, V)

Answer 41

1. coagulation pathway 2. bleeding 3. viral infections, bacteremia, malignancy, high dose chemo, vasculitis

Question 42

Nonimmune Platelet Destruction Disorders: 1. what 2 transfusions can be given as supportive treatment for nonimmune plt destruction and DIC? (P, P) 2. only effective treatment of DIC is to treat what? (UC) 3. pts with TTP or HUS should only receive plts for what? (LTB) 4. what is the potential harm of plt transfusion in pts with TTP or HUS? (IT, EOD) 5. should surgery be delayed when possible to control underlying disorder? 6. HUS in children can be managed without what but what may be necessary if severe renal failure? (P, D) 7. HELLP usually resolves after the delivery of what? (F) 8. pts who develop TTP like syndromes post partum need what? (PE)

Answer 42

1. plt, plasma 2. underlying cause 3. life-threatening bleeding 4. increased thrombosis, end-organ damage 5. yes 6. plasmapheresis, dialysis 7. fetus 8. plasma exchange

Question 43

autoimmune plt destruction disorders: 1. thrombocytopenia purpura can occur in adults post transfusion of what 2 things? 2. what 3 drugs can induce thrombocytopenic purpura? (Q, Q, S) 3. these drugs act as what and trigger what formation? (H, A) 4. these drugs serve as obligate molecules for antibodies to bind to what? (PS)

Answer 43

1. RBCs, plts 2. quinine, quinidine, sedormid 3. haptens, antibody 4. plt surface

Question 44

HIT: 1. type 1 appears how many days after heparin? 2. type 2 appears what day range after heparin? 3. what antibody for type 1? 4. what antibody for type 2? 5. can be treated with transfusion of what? 6. what can be given as an alternate to heparin? (B)

Answer 44

1. 1 day 2. 5-10 days 3. IgE 4. IgG 5. plts 6. bivalirudin

Question 45

Idiopathic thrombocytopenic purpura: 1. nonimmune, immune, or autoimmune? 2. is diagnosis made after all other cause of plt destruction are excluded? 3. continued high level of what is required to maintain chronically low plt count d/t shortened plt lifespan? (PP) 4. treated with high doses of what med type for 3 days? (C) 5. if emergency surgery or risk for intracranial hemorrhage, what 2 things can be given q 8-12 hrs? (I, P)

Answer 45

1. autoimmune 2. yes 3. plt production 4. corticosteroids 5. IVIG, plts

Question 46

Von willebrand's disease: 1. type 1 makes up what % of the disease? 2. the 3 types correlate with plasma levels of what 2 factors? 3. what med can be given to treat this? (D) 4. what does this med cause the release of from endothelial cells? (V) 5. what is the mcg/kg of this med? 6. this med has the best response in which of the 3 types of this disease? 7. this med immediately increases plasma levels of vWF and factor 8 enhancing function of what? (P)

Answer 46

1. 80% 2. vWF, factor 8 3. desmopressin 4. vWF 5. 0.3 mcg/kg 6. type 1 7. plt

Question 47

what are the 4 acquired plt function abnormalities? (MD, LD, U, D)

Answer 47

1. myeloproliferative disease 2. liver disease 3. uremia 4. dysproteinemia

Question 48

drugs that inhibit plt function: 1. 4 drugs and 1 drug type are strong associations (C, A, T, A; N) 2. 3 drug types are mild-moderate association (A, VE, FA) 3. 2 drugs, 2 drug types, and 1 substance are weak association (SNP, Q; OD, CD; A)

Answer 48

1. clopidogrel, aspirin, ticlopidine, abciximab, nsaids 2. antibiotics (high doses), volume expanders, fibrinolytic agents 3. sodium nitroprusside, quinidine; oncologic drugs, C/V drugs; alcohol

Question 49

what are the 6 NSAIDs that are strongly associated with inhibiting plt function? (P,P,I,I,N,K)

Answer 49

1. piroxicam 2. phenylbutazone 3. indomethacin 4. ibuprofen 5. naproxen 6. ketorolac

Question 50

Qualitative plt disordes and ansesthesia: 1. does absolute plt number predict bleeding risk? 2. what drug may improve mild to moderate plt defect if risk of bleeding is minor? (D) 3. what needs to be given if bleeding risk is greater? (P) 4. what 3 lab data can be measured? (BT, PFA, T) 5. plts dysfunction if body temp is less than what temp in celsius and less than what pH?

Answer 50

1. no 2. desmopressin 3. plts 4. bleeding time, plt function analysis, thromboelastogram 5. 35 degrees celsius, < 7.3

Question 51

Hemophilia A: 1. clinical severity is correlated with what factor level? 2. severe hemophilia has less than what % of normal level? 3. diagnosed at what time in life? (C) 4. these pts frequently need replacement with what factor? 5. the factor level of what % range of normal is enough to reduce severity of hemophilia?

Answer 51

1. factor 8 2. 1% 3. childhood 4. factor 8 5. 1-5%

Question 52

Hemophilia A and anesthesia: 1. when surgery is needed factor 8 levels need to be brought up near what %? 2. what is factor 8 half life in adults? (hours) 3. what is factor 8 concentrate half life in children? (hours) 4. infusion of factor 8 concentrate needs to be repeated q 8-12 hours to keep factor 8 level greater than what %? 5. if mild hemophilia, what med can be given? (D) 6. for dental procedures, what med type can be used and what are 2 meds of this med type? (A: A, TA)

Answer 52

1. 100% 2. 12 hours 3. 6 hours 4. >50% 5. desmopressin 6. antifibrinolytics; amicar, tranexamic acid

Question 53

Hemophilia B: 1. AKA what disease? (C) 2. what factor is of concern with this disease? 3. factor level less than what % of normal is associated with severe bleeding? 4. moderate disease is what % of normal? (range) 5. mild disease is what % of normal? (range) 6. recombinant or purified product of facor IX-prothrombin concentrates can be used to treat what type of bleeding episodes? (M) 7. what is factor 9 half-life? (hour, range) 8. repeat infusion with what % of original dose every 12-24 hours to keep factor 9 levels greater than what %?

Answer 53

1. christmas disease 2. factor 9 3. < 1% 4. 1-5% 5. 5-40% 6. mild 7. 18-24 hours 8. 50%, > 50%

Question 54

acquired factor 8 or 9 inhibitors: 1. what % range of hemophilia A pts are at risk to develop circulating inhibitors of factor 8? 2. prevalence is what % range in hemophilia B? 3. severe hemophilia-like syndrome can occur in genetically normal individuals d/t acquired waht against either factor 8 or 9? (A) 4. what study detects presence of inhibitors? (MS) 5. what 2 factor or what recombinant factor may be necessary?

Answer 54

1. 30-40% 2. 3-5% 3. autoantibodies 4. mixing study 5. factor 8, 9; recombinant factor 7a

Question 55

Do these need to be ABO compatible? 1. FFP 2. plts

Answer 55

1. yes | 2. no

Question 56

what 3 meds are antiplatelet? (P, N, T)

Answer 56

persantine, NSAIDs, thienopyridine

Question 57

HELLP: 1. what are the 3 conditions of this disease? 2. females develop this when they are what?

Answer 57

1. hemolysis, elevated liver enzymes, low plt count | 2. pregnant

Question 58

Plt count less than what is associated with these: 1. spontaneous bleeding 2. surgical bleeding risk 3. thrombocytopenia

Answer 58

1. 20,000 2. 50,000 3. 100,000

Question 59

These meds alter what lab value: 1. aspirin 2. lovenox 3. coumadin 4. NSAIDs 5. heparin

Answer 59

1. bleeding time 2. aPTT 3. PT 4. bleeding time 5. aPTT