Coagulation Disorders-COMP

Question 1

slide 10 Von willebrand disease

Answer 1

Factor VII deficiency

Question 2

Von willebrand disease is the

Answer 2

most common INHERITABLE coagulation disorder

Question 3

Hemophillia A Treatment

Answer 3

Plasma derived (may developed antibodies ) and recombinant factor

Question 4

1 unit of factor VIII is equal to

Answer 4

amount found in 1Ml of plasma

Question 5

For surgery you need_____concentrate

Answer 5

100% of concentrate

Question 6

Each unit of factor of VIII per kg of body weight raises plasma by

Answer 6

2%

Question 7

Tx for hemophilliac going through surgerry

Answer 7

Factor VIII levels 1 hour prior to surgery 100% then 50% wound healing begins, then 30% until complete healing

Question 8

Dose of Factor VIII

Answer 8

Concentrate 50 units/kg then dose q12h

Question 9

______infuse over 15 minutes may be given to treat mild bleeding episodes

Answer 9

• DDAVP 0.3-0.4ug/Kg

Question 10

Intranasal treatment for DDAVP what is recommended?

Answer 10

•Recommended to give a test dose prior to using intranasal DDAVP

Question 11

Most common side effect of DDVAP

Answer 11

FACIAL FLUSHING

Question 12

DDVAP also increase

Answer 12

Plasminogen level

Question 13

When do you suspect inhibitors

Answer 13

when calculated dose does not result in desired increase in factor level.

Question 14

Scenario: factors VIII inhibitors

Answer 14

FEVA (factor inhibitors treatment ) Bypass factor VIII

Question 15

Hemophillia A is due to a deficiency in what clotting factors factor?

Answer 15

Factor VIII deficiency

Question 16

Hemophillia B is due to a deficiency in what clotting factors factor?

Answer 16

Factor IX deficiency

Question 17

How are patients with Hemophilia classified

Answer 17

Clinical bleeding with the degre of deficiency
Severe hemophilliacs
Moderate hemophilliacs
Mild hemophilliacs

Question 18

Most commonly affected for hemophillia A

Answer 18

Males affected; females are carriers

Question 19

Hemophillia A presentation

Answer 19

joint or muscle hemorrhage with prolonged bleeding after trauma or surgery.

Question 20

Hemophillia A Diagnosed

Answer 20

normal PT and abnormal PTT (unique to intrinsic factors VIII, IX, XI and XII)

Question 21

Lab test for hemophillia A

Answer 21

Test for Von willebrand disease

Speficic factor assay for factor VIII or IX

Question 22

Recommended treatment for Hemophillia A

Answer 22

Severe or moderate: Cryoprecipitate or FACTOR VIII concentrate

Question 23

2 sources of factor VIII concentrate

Answer 23

Plasma derived

Recombinant Factor VIII

Question 24

1 unit of factor VIII is the amount found in

Answer 24

1 pooled plasma

Question 25

Treatment plan for hemophilliacs undergoing surgery? Factor level

Answer 25

Desired factors VIII levels 1 hour prior to surgery 100% | then 50% until wound healing begins then 30% until complete wound healing

Question 26

Treatment plan for hemophilliacs undergoing surgery? Dose factor VIII concentrate

Answer 26

50 units/kg Then dose 12 hr

Question 27

Treatment plan for hemophilliacs undergoing surgery?Perioperative and postop

Answer 27

Plan must be in place preop; evaluation for inhibitors crucial prior to elective surgery

Question 28

What is cryoprecipitate?

Answer 28

•Rich in factor VIII, fibrinogen, and von Willibrand’s factor

Question 29

Each bag of cryoprecipitate contains_______ Potency can vary from batch to batch

Answer 29

70-100 units of factor VIII activity per 10-20ml bag.

Question 30

What is Desmopressin Acetate (DDAVP) ?

Answer 30

Used to treat mild factor VIII deficiencies.

Question 31

Main action of DDAVP

Answer 31

transiently increases factor VIII and von Willibrand’s factor levels and shortens prolonged bleeding

Question 32

DDAVP is a

Answer 32

synthetic analog of the antidiuretic hormone vasopressin

Question 33

Dose of DDAVP for mild bleeding

Answer 33

0.3-0.4 mcg/Kg infuse over 15 minutes may be given to treat mild bleeding episodes

Question 34

Inhibitors : Treatment can be approached by different methods •

Answer 34

Reduce inhibitor levels with immunosuppresant therapy (cyclophosphamide), gamma-globulin, plasmopheresis Or by tolerance with high dose OR frequent administration of factor

Question 35

B vs Bm The exception is in hemophilia Bm in which

Answer 35

Both the PT and APTT are prolonged; Hemophillia B has normal PT

Question 36

What is the treatment of choice for Hemophilia B?

Answer 36

High purity factor IX plasma concentrate

Question 37

High purity factor IX plasma concentrate replace older products, known as ______ witch also contained _______________dependent cofactors responsible for thromboembolic complications associated with their use.

Answer 37

•Replaced older products, known as PCC witch also contained vitamin K dependent cofactors responsible for thromboembolic complications associated with their use.

Question 38

Each unit of factor IX infused per Kg increases factor IX plasma levels

Answer 38

1%.

Question 39

Classification of Von willebrand

Answer 39

quantitative or qualitative of von Willibrand’s factor

Question 40

Von willebrand disease results in both

Answer 40

both abnormal platelet function and defective plasma clotting.

Question 41

von Willebrand’s disease and it’s variants are defined by the type of abnormality in vWF: Factor VIII acAvity is usually low depending on the degree of vWF deficiency, however it may approach normal in some paAents.

Answer 41

quantitative, qualitative or both. •

Question 42

Diagnosis: VWF activity is measured by

Answer 42

1)the bleeding time and 2)ristocetin induced platelet aggregation (RIPA). •

Question 43

What is the recommended treatment for mild von willebrand disease? Goals?

Answer 43

•Goals of therapy are to improve factor VIII levels, and to improve bleeding time. •

Question 44

What is the recommended treatment for mild von willebrand disease?

Answer 44

Cryoprecipitate • The majority of commercial concentrates used to treat hemophilia A are not suitable for vW disease because they lack high molecular weight multimers, and therefore do not correct the bleeding time. •

Question 45

What is the recommended treatment for mild von willebrand disease that can achieve clinical hemostasis

Answer 45

Humate -P, Koate-HS, and Koate-HP have been reported to achieve clinical hemostasis prior to surgery and can be used in place of cryoprecipitate

Question 46

_________ dose and meds: usually induces a dose dependent increase in all factor VIII activity, with both factor VIII and vWF increasing four to six-fold

Answer 46

Intravenous DDAVP 0.3 to 0.4ug/Kg over 15 -30 minutes

Question 47

How long does the response to DDAVP lasts _____and should be repeated

Answer 47

The response usually last 4-8 hours. • The dose should be repeated in 12 hours. •

Question 48

When should DDAVP not be used?

Answer 48

should not be used before the type of vW disease is known

Question 49

DDVAP should not used before you know the type of DDVAP because?

Answer 49

In type 2B VW disease DDAVP can cause platelet aggregation and severe thrombocytopenia

Question 50

Use high molecular weight vWF because

Answer 50

they have the Highest RISTOCETIN FACTOR, most efficient for platelet adhesion