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SRNAPharmacology II > Coagulation Disorders-COMP > Flashcards

Coagulation Disorders-COMP Flashcards

1
Q

slide 10 Von willebrand disease

A

Factor VII deficiency

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2
Q

Von willebrand disease is the

A

most common INHERITABLE coagulation disorder

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3
Q

Hemophillia A Treatment

A

Plasma derived (may developed antibodies ) and recombinant factor

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4
Q

1 unit of factor VIII is equal to

A

amount found in 1Ml of plasma

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5
Q

For surgery you need_____concentrate

A

100% of concentrate

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6
Q

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

A

2%

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7
Q

Tx for hemophilliac going through surgerry

A

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

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8
Q

Dose of Factor VIII

A

Concentrate 50 units/kg then dose q12h

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9
Q

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

A

• DDAVP 0.3-0.4ug/Kg

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10
Q

Intranasal treatment for DDAVP what is recommended?

A

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

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11
Q

Most common side effect of DDVAP

A

FACIAL FLUSHING

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12
Q

DDVAP also increase

A

Plasminogen level

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13
Q

When do you suspect inhibitors

A

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

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14
Q

Scenario: factors VIII inhibitors

A

FEVA (factor inhibitors treatment ) Bypass factor VIII

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15
Q

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

A

Factor VIII deficiency

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16
Q

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

A

Factor IX deficiency

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17
Q

How are patients with Hemophilia classified

A

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

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18
Q

Most commonly affected for hemophillia A

A

Males affected; females are carriers

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19
Q

Hemophillia A presentation

A

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

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20
Q

Hemophillia A Diagnosed

A

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

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21
Q

Lab test for hemophillia A

A

Test for Von willebrand disease

Speficic factor assay for factor VIII or IX

22
Q

Recommended treatment for Hemophillia A

A

Severe or moderate: Cryoprecipitate or FACTOR VIII concentrate

23
Q

2 sources of factor VIII concentrate

A

Plasma derived

Recombinant Factor VIII

24
Q

1 unit of factor VIII is the amount found in

A

1 pooled plasma

25
Q

Treatment plan for hemophilliacs undergoing surgery? Factor level

A

Desired factors VIII levels 1 hour prior to surgery 100%

then 50% until wound healing begins then 30% until complete wound healing

26
Q

Treatment plan for hemophilliacs undergoing surgery? Dose factor VIII concentrate

A

50 units/kg Then dose 12 hr

27
Q

Treatment plan for hemophilliacs undergoing surgery?Perioperative and postop

A

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

28
Q

What is cryoprecipitate?

A

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

29
Q

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

A

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

30
Q

What is Desmopressin Acetate (DDAVP) ?

A

Used to treat mild factor VIII deficiencies.

31
Q

Main action of DDAVP

A

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

32
Q

DDAVP is a

A

synthetic analog of the antidiuretic hormone vasopressin

33
Q

Dose of DDAVP for mild bleeding

A

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

34
Q

Inhibitors : Treatment can be approached by different methods •

A

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

35
Q

B vs Bm The exception is in hemophilia Bm in which

A

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

36
Q

What is the treatment of choice for Hemophilia B?

A

High purity factor IX plasma concentrate

37
Q

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

A

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

38
Q

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

A

1%.

39
Q

Classification of Von willebrand

A

quantitative or qualitative of von Willibrand’s factor

40
Q

Von willebrand disease results in both

A

both abnormal platelet function and defective plasma clotting.

41
Q

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.

A

quantitative, qualitative or both. •

42
Q

Diagnosis: VWF activity is measured by

A

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

43
Q

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

A

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

44
Q

What is the recommended treatment for mild von willebrand disease?

A

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. •

45
Q

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

A

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

46
Q

_________ 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

A

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

47
Q

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

A

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

48
Q

When should DDAVP not be used?

A

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

49
Q

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

A

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

50
Q

Use high molecular weight vWF because

A

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

SRNAPharmacology II (32 decks)

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