112 Coagulation

Question 1

defined as strategy for maintaining the missing clotting factor level at about 1% of higher on a regular basis in order to prevent bleeds, esp the onset of hemarthrosis

Answer 1

primary prophylaxis

Question 2

how is primary prophylaxis done

Answer 2

regular infusion of FVIII (3 days/ week ) or FIX (2 days/ week)

Question 3

One unit of FVIII per KBW increases the plasma FVIII level by how much

Answer 3

2%

Question 4

True or false. FIX replacement are different from those for FVIII

Answer 4

true. because FIX recovery after infusion is usually only 50% as per predicted value

Question 5

what is the half life of FVIII

Answer 5

8-12 hours thus requires injections twice a day to maintain therapeutic levels

Question 6

what is the half of FIX

Answer 6

about 24 hours thus a once-a-day injection is sufficient

Question 7

enriched with FVIII protein

Answer 7

cryoprecipitate

Question 8

how much FVIII does each unit of cryoprecipitate contain

Answer 8

about 80 IU of FVIII

Question 9

synthetic vasopressin analog that causes transient rise in FVIII and vWF but NOT FIX

Answer 9

DDAVP

Question 10

how is DDAVP given

Answer 10

0.3 ug/KBW, over a period of 20 min is expected to raise FVIII by two to threefold over baseline, peaking between 30- 60 min after infusion

Question 11

how is antifibrinolytic drugs given to bleeding gums and GI in hemophiliacs

Answer 11

tranexamic acid 25 mg/kg 3-4x a day maximum loading load of 200 mg/kg or 1 gram followed by 100 mg/kg maximum 30 g/d every 6 hours

Question 12

true or false. tranexamic acid is not given to control hematuria as risk for occlusive clot in the lumen of the GUT

Answer 12

True.

Question 13

major complication of hemophilia treatment

Answer 13

formation of alloantibodies to FVIII or FIX

Question 14

laboratory tests required to confirm the presence of an inhibitor

Answer 14

APTT with a mix

Question 15

interpretation of mixing studies. normal? prolonged?

Answer 15

normal: hemophilia
prolonged: inhibitors

Question 16

most effective therapy in hemophiliacs with inhibitors

Answer 16

immune tolerance induction (ITI)

Question 17

most prevalent primary liver cancer in hemophiliac

Answer 17

hepatocellular cancer

Question 18

in hemophiliac carriers when is the risk for bleeding highest?

Answer 18

after delivery as clotting factors rapidly decrease; during pregnancy there is a threefold increase in FVIII

Question 19

How is clotting factor infusion done in to prevent imminent bleeding among patients carriers of hemophilia

Answer 19

infusion of factor concentrate to 50-70% level for 3 days in the setting of vaginal delivery and up to 5 days for cesarean delivery

Question 20

what is the half life of FXI

Answer 20

40- 70 hours

Question 21

how is FXI deficiency addressed

Answer 21

infusion of FFP at 15-20 ml/ k to maintain trough levels ranging from 10 to 20%

Question 22

clinicopathologic syndrome characterized by widespread intravascular thrombin fibrin formation

Answer 22

DIC

Question 23

common cause of DIC in pregnant women

Answer 23

abruptio placentae or amniotic fluid embolism

Question 24

severe form of DIC resulting from thrombosis of extensive areas of the skin

Answer 24

purpura fulminans

Question 25

central mechanism of DIC

Answer 25

uncontrolled generation of thrombin by exposure tof the blood to pathologic levels of tissue factor

Question 26

most sensitive test for DIC

Answer 26

FDP

Question 27

clinical scenarios with low grade compensated DIC

Answer 27

giant hemangioma, metastatic carcrinoma and death fetus syndrome

Question 28

True or false. Clotting factor concentrates are not recommended in controlling bleeding in DIC

Answer 28

True. due to limit efficacy and can further aggravate DIC

Question 29

what blood products are given in DIC

Answer 29

FFP or platelet concentrates

Question 30

most effective blood product to correct hemostasis in patients with liver failure

Answer 30

FFP

Question 31

most common target of antibody formation

Answer 31

FVIII