Cardio Lecture 4- Inherited/acquired Bleeding Disorders

Front 1

what deficiency causes hemophilia A

what deficiency causes hemophilia B

what deficiency causes von willebrand disease?

Back 1

A- factor VIII deficiency

B- factor IX (9) deficiency

vWD- vWF deficiency, which leads to a factor VIII deficiency

Front 2

how is hemophilia inherited genetically?

are hemp A and B clinically distinguishable?

Back 2

it is sex linked inheritance, (carried on the X chromosome), which means it skips generations and usually affects males (since most girls have 1 good X chromosome still, men get the 1 mutated X chromosome)

no, u have to run a test to tell the difference b/w A and B

Front 3

when do you expect a child has a bleeding disorder? adult? both?

Back 3

child- bleeding form umbilical stump, bleeding from circumcision, mucosal (nose) bleeds

adult- mennorhagia, >15 min nosebleed

both- family history of mucosal bleeding, muscle bleeds, joint bleeding, mennorhagia, early hysterectomy (removal of uterus0

Front 4

describe how bleeding is different among diseases

Back 4

bleeding will vary in FREQUENCY and it could be seasonal (i.e. kids play outside in summer more so might see more bruising then)

Front 5

what determines how severe hemophilia is?

Back 5

how deficient u are in the factor

mild...> 5%

1-5% - moderate

<1% severe

Front 6

some mild and moderate hemophilia A responds to which drug?

Back 6

DDAVP- a ADH analogue- causes FVIII to be released and vWF

Front 7

vWD is inherited in what manner?
where is hemorrhage usually seen in vWD?

Back 7

autosommal dominant, muscosal hemorrhage usually seen

Front 8

how do you treat vWD

Back 8

90% respond to DDAVP- releases F VIII and vWF

- transexamic acid (plasmin inhibitors...plasmin normally breaks down clots)

- vWF replacement

Front 9

name 2 subtypes of vWD...

hint: 2 disorders both increase affinity to Gp 1b, one disease involves a mutation on vWF, the other involves a mutation on the platelet receptor for vWF

Back 9

vWD type IIb- a mutation in vWF which increases affinity to GP1b

platelet or pseudotype- mutation in GP1b which increases affinity for vWF

Front 10

how do u treat vWD subtypes?

Replace what is abnormal,

therefore how would u treat IIB, pseudo type?

Back 10

IIb- treat with functional vWF, since in IIb the person has mutated vWF that doesn't work

pseudo- treat with platelets

Front 11

what are the PT and PTT tests like for hemophilia A and B? which one is normal

are the Bleeding times normal in hemophilia?

hint: think wheat factors cause their deficiency and whether these factors are in the intrinsic or extrinsic pathway

Back 11

in A and B the PTT test is prolonged, the PT is normal

explanation: people with hemophilia A and B are deficient in factors 8 and 9 respectively (factors 8 and 9 are in the intrinsic pathway which is what PTT measures)...therefore is is abnormal upon testing

yes, bleeding times are normal

Front 12

what are the PT and PTT tests like for people with vWD? bleeding time?

Back 12

PT and PTT are both normal, bleeding time is abnormal.

Bleeding time is prolonged

Front 13

will people with vWD have their platelets aggregate together when ristocetin (causes platelet aggregation) is added? what about subtypes of vWD?

Back 13

in regular vWD, risto can't make the platelets aggregate

in subtypes of vWD, risto makes platelets aggregate

Front 14

how do u treat hemophilia A?

Back 14

DDAVP, recombinant factor VIII, plasma derived factor VIII


- makes sense to give F VIII since people with hemo A are deficient in F VIII

Front 15

how do u treat hemophilia B?

do you give DDAVP?

Back 15

treat with- plasma Factor IX, or recombinant factor IX

no, you do not give DDAVP.

Front 16

how do u treat vWD?
do u treat all vWD subtypes with DDAVP?

Back 16

treat with DDAVP, transexamic acid, platelet derived vWF

no, u do not treat IIb or pseudo vWD with DDAVP

Front 17

who do you often see vitamin K deficiency in? why?

rx? what route?

Back 17

hospital patients...antibiotics dec vit k production by gut flora and their diet often lacks bit k


1-10 mg of vit k,
orally (best since it goes straight to liver where its effective)
IV, or subcutaneous ok
never IM or u will get clots

Front 18

in vit k deficiency, will clotting factors still show up on a person's immune assay test? are they functional?

Back 18

yes, they will not show up BUT they will NOT be functional since there is no vit K.

Front 19

name 2 mechanisms of Disseminated intravascular Coagulation

Back 19

1) intravascular of thrombin activates plasmin (so u have little clots floating around with plasmin trying to break them down)

2) intravascular activation of plasmin by annexin

Front 20

how do u treat DIC caused by intravascular activation of thrombin and plasmin? what don't you use? what should u never use and why?

Back 20

treat the underlying cause i.e. cancer, trauma, etc

u can use blood products WITH heparin

never use plasmin inhibitors or there is no way for the clots to be broken down...your patient gets smacked by them and dies

Front 21

you see a patient with DIC caused by intravascular activation of plasmin by annexing...what disease is this commonly seen in? treatment?

can you use plasmin inhibitors?

Back 21

promyelocytic leukemia

treat underlying cause

yes, u can use plasmin inhibitors (i.e. transexamic acid) since mini thrombin clots aren't floating around in this type of DIC.

Front 22

antidote for heaprin/ LMWH/ Dalbigatran (NOAC)/ Rivaroxaban

asa/ NSAID/ Anti GP IIb/IIIa?

Back 22

heparin= protamine, LMWH = protamine 60%,

dalbigatran (aPCC), RivaroXapan- PCC

asa- platelets, NSAID- platelets/time

Anti GP 2b3a- non reversible so platelets

Front 23

how does dypridamole work? is it reversible?

Back 23

inhibits interaction with ADP and functions by interfering via AMP by inhibiting phosphodiesterase

yes reversible

Front 24

order the drugs Clopidogrel presuragel, Ticagrelor in order from best to worst... which ones are reversible

Mechanism of action?

Back 24

Ticagrelor (best) --> presuragel --> clopidogrel

only ticagrelor reversible
block ADP receptor.