vWF, hemophilia, DIC, antithrombin deficiency,

Question 1

where is vWF synthesized

Answer 1

in vascular endothelium and megakaryocytes

Question 2

two key functions of vWF

Answer 2

anchors platelet to vessel wall at the site of vascular injury (platelet adhesion)
carries activated factor 8 in plasma

Question 3

type 1 vWF disease

Answer 3

mild to moderate reduction in amount of vWF produced

Question 4

type 2 vWF disease

Answer 4

vWF produced doesn’t work well

Question 5

type 3 vWF disease

Answer 5

severe reduction in amount of vWF produced

Question 6

what labs are affected by vWF disease?

Answer 6

no change in PT/IN, platelet count, fibrinogen
increase in PTT and bleeding time!

Question 7

how does DDAVP help with vWF disease (and which type responds best)

Answer 7

its a synthetic ADH that stimulates release of vWF and increases facto 8 activity.
therefore, type 1 responds best and type 3 doesn’t respond at all

Question 8

DDAVP dose

Answer 8

.3mcg/kg IV. bleeding time improved for 12-24h

Question 9

SE of DDAVP

Answer 9

vasodilation - HoTN with rapid administration and hyponatremia due to free water retention

Question 10

cryo contains which factors and can be used for which vWF disease types

Answer 10

contains factors 8, 13, fibrinogen
can be used for type 1, 2, or 3

Question 11

ffp contains which factors and cab be used for which vWF disease types

Answer 11

clotting factors. can also be used for vWF 1, 2, 3 but there are better alternatives like cryo

Question 12

purified 8vWF concentrate is used for which disease type

Answer 12

1st line for vWF type 3 disease, decreases risk of transfusion

Question 13

which of the two hemophilias is more severe

Answer 13

A is more severe than B

Question 14

what happens to PTT and PT with hemophilia A and B patients?

Answer 14

PTT is prolonged but PT is normal

Question 15

treatment for hemophilia A

Answer 15

factor 8 concentrate. t1/2 is 8-12h, continue 2-6w past surgery

Question 16

hemophilia A is a deficiency of which factor

Answer 16

8

Question 17

hemophilia B is a deficiency of which factor

Answer 17

9

Question 18

treatment for hemophilia B

Answer 18

factor 9 prothrombin complex, t1/2 18-24h

Question 19

what is factor 9 prothrombin complex associated with?

Answer 19

thromboembolic compications

Question 20

which factor is a treatment for both hemophilia a and b and what are the associated risks

Answer 20

recombinant factor 7 (“bypasses” 8 and 9), risk of arterial and venous thromboses.
90-120mcg/kh

Question 21

recombinant factor 7 is also used for last ditch tx of

Answer 21

bleeding without ID’d cause- 20-40mcg/kg

Question 22

signs of DIC

Answer 22

ecchymosis, petechiae, mucosal bleeding, bleeding at IV sites,
prolonged PT and PTT
increased D dimer and fibrin split products
decreased fibrinogen and antithrombin

Question 23

tx of DIC

Answer 23

hypovolemia: IVF
coagulopathy: FFP, platelets, cryo
microvascular thrombosis: IV heparin or LMWH

Question 24

antithrombin inactivates factors

Answer 24

9, 10, 11, 12 which ultimately leads to thrombin (factor 2a) inhibiton

Question 25

patients with antithrombin deficiency are unresponsive to

Answer 25

heparin

Question 26

treatment of antithrombin deficiency includes

Answer 26

antithrombin concentrate, FFP

Question 27

HIT causes

Answer 27

clot formation

Question 28

type 1 HIT MOA and cause

Answer 28

HIT inducted platelet aggregation after large heparin dose

Question 29

how many days after heparin admin does type 1 HIT occur

Answer 29

1-4 days after administration

Question 30

platelet count during type 1 HIT

Answer 30

<100,000

Question 31

tx for type 1 HIT

Answer 31

resolves spontaneously even if heparin is continued

Question 32

type 2 HIT MOA and cause

Answer 32

antiplatelet antibodies (IgG) attack factor 4 immune complex platelet aggregation. occurs after any heparin dose

Question 33

how many days after heparin admin does type 2 HIT occur

Answer 33

5-14 days

Question 34

platelet count during type 2 HIT

Answer 34

<50k

Question 35

treatment for type 2 HIT

Answer 35

decrease heparin and use anticoagulants with direct thrombin inhibition (biialrudin, hiruden, argatroban)

Question 36

a deficiency of protein C or S can produce

Answer 36

hyper coagulable state

Question 37

thromboembolism is treated with

Answer 37

heparin then transitioned to warfarin

Question 38

factor 5 leiden causes resistance to

Answer 38

anticoagulant effects of protein C

Question 39

is lifelong anticoagulation necessary for someone who has factor 5 leiden

Answer 39

no unless theyre experiencing alot of thrombotic events

Question 40

patho of sickle cell disease

Answer 40

valine is substituted for glutamic acid on beta globulin chain which alters RBC geometry

Question 41

describe vaso occlusive criis

Answer 41

sickled cells impair tissue perfusion and create ischemic injury.
tx is analgesics and hydration

Question 42

hydroxyurea in the setting of vaso occlusive crisis

Answer 42

decreases incidence and severity

Question 43

describe acute chest syndrome

Answer 43

caused by thrombosis, embolism, infection
common postoperatively, caused by hypoventilation, narcotics, splinting, pain

Question 44

describe sequestration crisis

Answer 44

occurs when spleen removes RBC’s from circulation at a faster rate than bone marrow produces them
leads to anemia and hemodynamic instability

Question 45

describe aplastic crisis

Answer 45

RBC with HGBs have short t1/2 so even a small amount of bone marrow suppression can cause anemia. usually r/t viral infection like parvo B19

Question 46

two co existing diseases that are seen with a patient who has sickle cell

Answer 46

asthma (50%) and pHTN (10%)

Question 47

describe sickle cell trait

Answer 47

heterozygous, usually does not advance to crisis unless sever hypoxemia occurrsr