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3rd shift Topics > coagulation > Flashcards

coagulation Flashcards

1
Q

The process by which a blood clot forms to reduce
blood loss after damage to a blood vessel

A

coagulation

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2
Q

group of diseases caused by deficiency of clotting factors which may lead to defects in normal clot formation process

A

Coagulation disorder

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3
Q

A process by which the body repairs damage to a blood vessel to
prevent hemorrhage

A

Hemostasis

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4
Q

Two general steps of hemostasis

A
  • platelet plug formation
  • fibrin clot formation
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5
Q

At the site of injury, a hemostatic plug is formed by the interaction of the blood vessels, platelets and coagulation factors

A

Platelet plug formation

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6
Q

As the plug is formed, the coagulation system generates thrombin from prothrombin, initiating fibrin clot formation

A

Fibrin clot formation

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7
Q

Process of hemostasis

A
  1. injury
  2. vascular spasm
  3. platelet plug formation (primary hemostasis
  4. coagulation cascade (secondary hemostasis)
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8
Q

A blood vessel is severed. Blood and blood components (e.g., erythrocytes, white blood cells, etc.) are leaking out of the breaks.

A

Injury

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9
Q

The smooth muscle in the vessel wall contracts near the injury point, reducing blood loss.
○ inflammation

A

vascular spasm

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10
Q

Platelets are activated by chemicals released from the injury site and by contact with underlying collagen. The platelets become spiked and stick to each other and the wound site.

A

Platelet plug formation (Primary Hemostasis)

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11
Q

In coagulation, fibrinogen is converted to fibrin, which forms a mesh that traps more platelets and erythrocytes, producing a clot.

A

Coagulation Cascade (Secondary Hemostasis

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12
Q

Contact Activation Pathway

A

Intrinsic pathway

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13
Q

Tissue factor pathway

A

extrinsic pathway

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14
Q

Factor X

A

Final common pathway

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15
Q

if there is a presence of damage, Factor XI will become XIa → activation of IX to IXa, with VIIIa → activation of X to Xa

A

Intrinsic pathway

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16
Q

Factor III to IIIa → activation of VII and VIII → combine with III to activate Factor X to Xa → activation of Va → Prothrombin → thrombin → fibrinogen → fibrin

A

Extrinsic pathway

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17
Q

surface damage

A

Intrinsic pathway

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18
Q

in trauma + inflammation

A

extrinsic pathway

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19
Q

Initiated through the activation of two separate pathways of intrinsic and extrinsic pathways

A

Coagulation cascade

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20
Q

both pathways lead to the production of ________________ which marks the beginning of the common pathway of coagulation leading to cloth formation

A

Factor X

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21
Q

● Extrinsic pathway first, then intrinsic pathway
● Amplify the coagulation process

A

cross-activation

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22
Q

● Also known as Coagulation Factors
● Prompt reactions that activates more clotting or coagulation factors

A

Clotting factors

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23
Q

● Secreted primarily by the _____ and the ________
(Clotting factors)

A

liver and platelets

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24
Q

Vitamin K deficiency leads to:

A

bleeding

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25
Q

● The liver requires _________ as a co-factor to produce clotting factors

(Clotting factors)

A

Vitamin K

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26
Q

There are ____ clotting factors

A

12

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27
Q

There is no factor _____; it is not used already

A

VI

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28
Q

Inactivated forms:

(Clotting factors)

A

I, II, III

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29
Q

Activated forms:

A

Ia, IIa, IIIa

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30
Q

VI is identical to V (t or f)

A

T

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31
Q

Pathways are dependent to:

A

Clotting factors, calcium ions, and vitamin K

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32
Q

○ activated by damage directly to the blood vessels and exposure to collagen and circulating platelets in blood

A

Intrinsic pathway

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33
Q

○ activated by tissue damage, malignancy, inflammation, sepsis

A

Extrinsic pathway

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34
Q

Fibrinogen

A

I

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35
Q

Prothrombin

A

II

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36
Q

Tissue thromboplastin or tissue factor

A

III

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37
Q

Calcium ions

A

IV

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37
Q

Proaccelerin; labile factor

A

V

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38
Q

Antihemolytic factor A

A

VIII

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39
Q

Not used

A

VI

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40
Q

Proconvertin; stable factor

A

VII

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41
Q

Antihemolytic factor B (plasma thromboplastin component); Christmas factor

A

IX

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41
Q

Hageman factor

A

XII

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42
Q

Fibrin-stabilizing factor

A

XIII

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42
Q

Stuart-Prower factor (thrombokinase)

A

X

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43
Q

antihemolytic factor C (plasma thromboplastin antecedent)

A

XI

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44
Q

Common; converted into fibrin

A

I

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45
Q

Extrinsic (1)

A

Tissue thromboplastin/ tissue factor

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46
Q

Common; converted into thrombin

A

II

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46
Q

Extrinsic and Intrinsic (1)

A

V

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47
Q

entire process

A

IV

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47
Q

Not used

A

VI

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48
Q

Extrinsic (2)

A

Proconvertin

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49
Q

Intrinsic; deficiency results in hemophilia A

A

VIII

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50
Q

Intrinsic; deficiency results in hemophilia B

A

IX

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51
Q

Extrinsic and intrinsic (2)

A

X

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52
Q

Intrinsic; deficiency results in hemophilia C

A

XI

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53
Q

Intrinsic; initiates clotting in vitro also activates plasmin

A

XII

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54
Q

Stabilizes fibrin; slows fibrinolysis

A

XIII

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55
Q

Risk factors for Coagulation disorders

A
  • age
  • family history and genetics
  • medical conditions
  • medications
  • sex
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56
Q

______ are more likely to develop Vit K deficiency

A

newborns

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57
Q

risk factors medications

A

● antibiotics
● blood thinners
● anticoagulants

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58
Q

● blood transfusion
● bowel diseases (IBD) or bowel surgery
● cancer
● congenital heart disease
● hypothyroidism
● some autoimmune diseases

A

medical conditions

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59
Q

Sex: congenital hemophilia

A

males

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60
Q

acquired hemophilia during and after pregnancy

A

females

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61
Q

Signs and symptoms of coagulation disorders

A

● Blood in urine or stool
● Excessive bleeding
● Frequent, large bruises
● Heavy bleeding after giving birth
● Heavy menstrual bleeding
● Petechiae
● Redness, swelling, stiffness or pain
● Umbilical stump bleeding

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62
Q

○ Does not stop even after applying pressure
○ Spontaneous bleeding
■ Nose bleed
■ Bleeding after dental procedure or surgery

A

● Excessive bleeding

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63
Q

○ More than 7 days or change of sanitary pads every hour

A

● Heavy menstrual bleeding

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64
Q

○ Bleeding under the skin causing tiny spots
○ Violet or brown

A

Petechiae

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65
Q

○ Bleeding after 1-2 weeks after the umbilical cord was cut

A

Umbilical stump bleeding

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66
Q

Complications

A

● Bleeding in the brain or CNS
○ Hemorrhagic stroke
● Bleeding in the throat
○ Inflammation → swelling → DOB
● Bleeding into the abdomen
● Damaged joints
● Hard masses in the bones
● Miscarriages

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67
Q

Diagnosis

A
  • CBC
  • PTT
  • PT
  • Mixing test
  • vWF
  • Clotting factor test
  • Bethesda test
  • Factor XIII antigen and activity assays
  • Genetic testing
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68
Q

● measure different parts of the blood and identify the number of blood cells and platelets

A

CBC

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69
Q

● how long it takes for blood to clot by determining the clotting factors involved

A

PTT

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70
Q

● same with PTT but also measures the clotting factors not covered by PTT

A

PT

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71
Q

PTT measures

A

Intrinsic

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72
Q

PT measures

A

Extrinsic

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73
Q

● identify whether the bleeding is caused by antibodies blocking the functions of the clotting factors
● If autoimmune

A

Mixing test

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74
Q

● measure the amount of vWF and if these factors are working correctly

A

von Willebrand factor (vWF) tests

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75
Q

● determine if clotting factors are absent or present below normal levels

A

Clotting factor tests

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76
Q

● to check for antibodies for Factors XIII and IX

A

Bethesda test

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77
Q

● Factor XIII deficiency

A

Factor XIII antigen and activity assays

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78
Q

● to identify if genes are responsible for coagulation disorders

A

Genetic testing

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79
Q

platelet function analyzer

A

PFA-100

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80
Q

Laboratory Monitoring

A
  • Activated Partial Thromboplastin Time (aPTT)
  • Prothrombin Time (PT)
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81
Q

● Test for intrinsic and common pathways
● Dependent on activity of all coagulation factors, except for Factors VII and XIII

A

aPTT

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82
Q
  • 30 to 40 seconds
    ● Monitor heparin treatment and screen for hemophilia
    ● Integrity of intrinsic system (incl. XII, XI, VIII and IX)
    ● Liver disease decrease the production of these factors because liver is the main source
A

aPTT

83
Q

● Test for extrinsic pathway activity and common pathways (VII, V, X, prothrombin and fibrinogen)
● Measures Vitamin K-dependent factors activity (Factors II, VII, IX, and X)

A

PT

84
Q
  • 10 to 14 seconds
    ● Thromboplastin + Calcium to plasma = clotting time
    ● Liver disease and warfarin therapy
    ● Liver is the site of synthesis of plasma clotting factors
A

PT

85
Q

aPTT is dependent on all coagulation factors except:

A

VII and XIII

86
Q

PT: Test for extrinsic pathway activity and common pathways namely:

A

VII, V, X, prothrombin and fibrinogen

87
Q

PT: measures Vitamin K-dependent factors activity namely:

A

II, VII, IX, and X

88
Q

components measured of Bleeding Time:

A

Platelet function
Vascular integrity

89
Q

components measured of PT

A

I, II, V, VII, IX, X

90
Q

components measured of PTT

A

I, II, V, VIII, IX, X, XI,
XII

91
Q

components measured of thrombin time

A

I, II

92
Q

normal values of bleeding time

A

3 to 10 mins

93
Q

normal values of PT

A

10 to 14 secs

94
Q

Classification of Clotting Disorders

A
  • Congenital
  • acquired
94
Q

normal values of PTT

A

30 to 40 secs

95
Q

normal values of thrombin time

A

12-20 secs

96
Q

Congenital

A
  • hemophilia A
  • hemophilia B
  • von Willebrand’s disease
97
Q

○ A deficiency of factor VIII, inherited as an x-linked recessive trait affecting males, females being the carriers

A

hemophilia A

98
Q

Acquired

A
  • secondary to drugs
  • disease related
99
Q

○ The second most common inherited bleeding disorder after vWD

A

hemophilia A

100
Q

Males
(Hemophilia A)

A

hemizygous

101
Q

Females
(hemophilia A)

A

heterozygous

102
Q

○ Caused by a mutation of the factor IX gene, leading to factor IX deficiency

A

hemophilia B

103
Q

○ The second most common form of hemophilia, rarer than hemophilia A

A

hemophilia B

104
Q

Hemophilia B is also called:

A

royal blood’s disease

105
Q

Acquired: Disease-related

A

Liver disease, vitamin K deficiency, disseminated intravascular coagulation (DIC), fibrinolytic disorders

105
Q

Acquired: secondary to drugs

A

heparin, coumarin

106
Q

○ Microvascular and macrovascular clotting and compromised blood flow that is a rare but serious condition that causes abnormal blood clotting throughout the body’s blood vessels

A

Disseminated intravascular coagulation

107
Q

An inherited X-linked recessive and lifelong blood disorder where an essential blood clotting factor is either partly or completely missing

A

Hemophilia

108
Q

Hemophilia A is linked to level of ____________

A

Factor VIII

109
Q

1% _________ Hemophilia A

A

severe

110
Q

1%-5% __________ Hemophilia A

A

moderate

111
Q

6% - 30% _________ Hemophilia A little risk of spontaneous bleeding

A

Mild

112
Q

Management of Hemophilia

A

Factor replacement therapy

113
Q

● Main treatment of hemophilia
● Clotting factors from donations or made in the laboratory

A

Factor replacement therapy

114
Q

Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are slowly dripped or injected into a ________

A

vein

115
Q

Plasma-derived Factor Concentrates

A
  • cryoprecipitate and fresh frozen plasma (FFP)
116
Q

FFP: 250 - 300 mL, Cryoprecipitate:
(volume)

A

Cryoprecipitate: 10-20mL

117
Q

FFP: 30 minutes, Cryoprecipitate:
(time to prepare)

A

Cryoprecipitate: 30 minutes

118
Q

FFP: All, including factors II, VII, VIII, IX, X, XI, and vWF, Cryoprecipitate:
(other coagluation factors)

A

Cryoprecipitate: Factors VIII, XIII, and vWF

119
Q

FFP: 700 - 800 mg, Cryoprecipitate:
(Fibrinogen)

A

Cryoprecipitate: 150 - 250 mg

120
Q

○ Developing antibodies (proteins) that attack the clotting factor
○ Developing viral infections from human clotting factors
○ Damage to joints, muscles, or other parts of the body resulting from delays in treatment

A

Complications of Replacement Therapy:

121
Q

○ Very expensive, often to be imported
○ High risk of contamination with hepatitis B virus, hepatitis C virus, and HIV from the large number of donors

A

Problems with treatment with commercial factor VIII concentrate

122
Q

○ Recombinant Factor VIII concentrate, which does not come from human plasma
○ Genetically engineered using DNA technology and commercially prepared factor concentrates treated to remove or inactivate blood-borne viruses
○ No plasma or albumin, does not spread blood-borne viruses

A

Recombinant Factor Concentrates

123
Q

● FRT not needed; Desmopressin acetate may be given to raise the body’s level of Factor VIII

A

Mild hemophilia
● Mild or no risk of spontaneous bleeding

124
Q

● FRT given at home two or three times a week; Preventive therapy usually is started in patients at a young age and may need to continue for life

A

Severe hemophilia
- maintenance

124
Q

● FRT only when bleeding occurs or to prevent bleeding that could occur when doing certain activities

A

Moderate Hemophilia
- only if there is bleeding

125
Q

● also known as ACE 910 or Emicizumab

A

Hemlibra

126
Q

Other treatment options:

A
  • Hemlibra
  • DDAVP or Stimate (Desmopressin Acetate)
  • Amicar
126
Q

● Desmopressin Acetate

A

DDAVP or Stimate (Desmopressin Acetate)

127
Q

● Epsilon Amino Caproic Acid

A

Amicar

128
Q

● It works by replacing the function of factor VIII, rather than replacing the missing clotting factor VIII directly

A

Hemlibra

129
Q

● These are medications that are similar to a hormone that occurs naturally in the body. The medications release factor VIII from where it is stored in the body tissues.
● Give ____________ for mild ONLY
○ Not FRT immediately, only for moderate and severe

A

DDAVP or Stimate

130
Q

● Medication that can be given through a vein or by mouth (as a pill or a liquid)
● It prevents blood clots from breaking down, resulting in a firmer clot, and is often used for bleeding in the mouth or after a tooth has been removed because it blocks a substance found in the saliva that breaks down clots.

A

Amicar

130
Q

New Hemophilia Agents: Treatment for Hemophilia A

A
  • Eloctate
  • Obizur
  • Nuwiq
  • Kovaltry
  • Afstyla
131
Q

New hemophilia Agents: Treatment for Hemophilia B

A
  • Rixubis
  • Alprolix
  • Idelvion
132
Q

● Approved June 2014
● Factor VIII recombinant; Fe fusion protein

A

Eloctate

133
Q

● Approved October 2014
● Recombinant

A

Obizur

134
Q

● Approved September 2015
● Recombinant factor VIII

A

Nuwiq

135
Q

● Approved March 2016
● Recombinant
● Use infusion line provided with product as it has in-line filter

A

Kovaltry

136
Q

● Approved May 2016
● Recombinant

A

Afstyla

137
Q

● Approved March 2014
● Recombinant; Fe fusion protein

A

Alprolix

138
Q

● Approved June 2013
● Recombinant factor IX

A

Rixubis

139
Q

● Approved March 2016
● Recombinant; albumin fusion protein

A

Idelvion

140
Q

Priorities in hemophilia treatment and care include:

A
  1. prevention of bleeding and joint damage
  2. prompt management of bleeding episodes including physical therapy and rehabilitation after joint bleeds
  3. pain management
  4. management of musculoskeletal complications
  5. prevention and management of inhibitors
  6. management of comorbidities
  7. dental care
  8. quality-of-life assessments and psychosocial support
  9. genetic counselling and diagnosis
  10. ongoing patient/family caregiver education and support
141
Q

● Acute bleeds should be treated __________________

(Principal care)

A

as quickly as possible

142
Q
  • Desmopressin administration can raise FVIII level ______________________ to control
    bleeding
A

adequately (3 to 6 times the baseline level)

143
Q

● ________ and ________ should be avoided
- could prolong bleeding, as it also inhibits platelet formation
○ Use paracetamol instead

A

ASA and NSAID

144
Q

● _____________ prevents periodontal disease and dental caries, which predispose to gum bleeding

A

Good oral hygiene

145
Q

General recommendations

A
  • Prophylaxis with clotting factor concentrates (CFCs)
  • Episodic replacement therapy
146
Q

● Regular replacement therapy or the regular intravenous (IV) infusion of the missing clotting factors
● FVIII in hemophilia A and FIX in hemophilia B

A

Prophylaxis with clotting factor concentrates (CFCs)

147
Q
  • On-demand therapy, the administration of CFCs only at the time of a bleeding episode
A

Episodic replacement therapy

148
Q

● _____________ is the standard of care for people with severe hemophilia, and for some people with moderate
hemophilia

(WFH Recommendations)

A

Prophylaxis

148
Q

● Both_____________ and _____________, as well as other hemostasis products when
appropriate, can be used for treatment of bleeding and prophylaxis in people with hemophilia

(WFH Recommendations)

A

virus-inactivated plasma-derived and recombinant CFCs

149
Q

● The use of cryoprecipitate can only be justified in situations where _________________________ as there is no proven advantage for their use over CFCs

(WFH Recommendations)

A

clotting factor concentrates are not available

150
Q

● It is strongly encouraged that _________________ procedures be used, if available

(WFH Recommendations)

A

viral-inactivation

151
Q

● 1-deamino-8-D-arginine vasopressin
● Synthetic analogue of vasopressin that boosts plasma levels Factor VIII and vWF

A

Desmopressin (DDAVP)

152
Q

● May be the treatment of choice for patients with mild or moderate hemophilia A when Factor VIII can be raised to an appropriate therapeutic level

A

Desmopressin (DDAVP)

153
Q

Serious sites of bleeding in hemophilia

A
  • Joints
  • Muscles, especially deep compartments (iliopsoas, calf, forearm)
  • Mucous membranes of the mouth, nose, and genitourinary tract
154
Q

Frequency of bleeding sites: 70% - 80%

A

Joints
- more common in hinged joints; ankles knees, elbows
- less common in multi-axial joints; shoulders, wrists, hips

154
Q

Life-threatening

A
  • Intracranial
  • Neck/throat
  • gastrointestinal
155
Q

Frequency of bleeding sites: 10% - 20%

A

Muscles

156
Q

Frequency of bleeding sites: 5% - 10%

A

Other sites, major bleeds

157
Q

Frequency of bleeding sites: <5%

A

CNS

158
Q

People with hemophilia with a ______ or _________ bleed, the WFH recommends following the PRICE principles in addition to increasing factor levels

A

muscle or joint bleed

159
Q

PRICE

A
  • Protection
  • Rest
  • Ice
  • Compression
  • Elevation
160
Q

● Reduce weight bearing or stress on the affected joint or muscle by using crutches or other supports such as a ‘collar and cuff’ for the arm
● Avoid putting weight on the affected side completely for the first 48 hours and possibly longer if it is a severe bleeding

A

Protection

161
Q

● The affected area should initially be rested completely, generally for the first 24 to 48 hours depending on the severity of the bleeding episode to allow the swelling to go down and prevent further bleeding
● The injured area should not be forced into any position
● ‘Rest’ is not the same as ‘immobility’, too much rest can also be damaging, so start to move the joint gently, as pain allows, within one or two days of treatment

A

Rest

162
Q

● Reduces swelling, and therefore pain
● If the muscle or joint is very swollen, wait a day or so before using a compression bandage and then apply as
pain allows
● Nurse or physiotherapist can provide an elasticated bandage, make sure it fits correctly as additional damage can be caused where the bandage is too tight
● Try not to allow wrinkles in the bandage and make sure to remove it at night before you go to sleep

A

Compression

162
Q

● Helps reduce swelling, prevent further bleeding, and eases pain
● Use a gel cold pack or an ice pack from a bag of frozen peas or crushed ice wrapped in a cloth
● Cold wraps or packs should be applied to the affected area for around 10 to 15 minutes every 2 hours or so
● Continue using ice for several days after the bleed if joint is still warm and swollen
● Do not apply for more than 20 minutes at a time
● Do not place ice directly on the skin as it can burn

A

Ice

163
Q

● Helps reduce swelling and relieve pain by increasing the blood flow away from the injured area
● The injured area should be raised above the level of the heart
● When elevating leg, remove the compression stocking to allow normal, healthy circulation.
● Elevate ‘little and often’ for around 20 minutes at a time

A

Elevation

164
Q

For people with hemophilia, the WFH recommends the use of antifibrinolytic drugs (____________________) alone or as adjuvant treatment, particularly in controlling mucosal bleeds and for invasive ___________

A

tranexamic acid, epsilon aminocaproic acid [EACA]; dental procedures

165
Q

● An antifibrinolytic agent
● Competitively inhibits the activation of plasminogen to plasmin
● Promotes clot stability

A

Tranexamic acid

166
Q

● ________________ for some types of hemophilic bleeding

(Tranexamic acid)

A

Adjunctive therapy

167
Q

● Useful for treating superficial soft tissue and mucosal bleeds (_________________)

(Tranexamic acid)

A

oral bleeding, epistaxis, and menorrhagia

168
Q

● Brand name:

(Tranexamic acid)

A

Hemostat

169
Q

Paracetamol/acetaminophen: if not effective

(pain management)

A

Switch to
- COX 2 inhibitors (celecoxib, meloxicam, nimesulide, and others)
- Paracetamol/ acetaminophen plus codeine (3-4 times a day)
- Paracetamol/acetaminophen plus tramadol (3-4 times a day)

170
Q

use a slow release product with an escape of a rapid release. Increase the slow release product if the rapid release product is used more than 4 times/day

(pain management)

A

Morphine

171
Q

should be used with caution in patients with hypertension and renal dysfunction (paint management)

A

COX-2

172
Q

○ Regular high-dose infusions of Factor VIII and Factor IX
○ Demanding and costly but provides 60-80% success chance

A

immune tolerance induction (ITI) therapy

173
Q

Eradication of inhibitors is currently best achieved through:

(Management of patients with inhibitors)

A

immune tolerance induction (ITI) therapy

174
Q

● For patients with hemophilia A with an inhibitor, the WFH recommends that ______________ should be used for regular prophylaxis in addition to clotting factor concentrates (CFC)

A

emicizumab

175
Q

Mild - moderate

(Factor Requirement Calculations)

A
  • soft tissue
  • muscles
  • hemarthrosis epistaxis
176
Q

Severe

(Factor Requirement Calculations)

A
  • CNS
  • GI
  • Neck/throat
177
Q

Up to 100%

(Factor Requirement Calculations)

A

Severe

178
Q

Up to 50%

(Factor Requirement Calculations)

A

Mild- moderate

179
Q

Type of Bleeding Episodes:
Early hemarthrosis, minor muscle or oral bleeding

(Desired Factor VIII Increase)

A

Mild

180
Q

Type of Bleeding Episodes:
Muscle bleeding, bleeding into the oral cavity or mild head trauma

(Desired Factor VIII Increase)

A

Moderate

181
Q

Type of Bleeding Episodes:
Life or limb threatening hemorrhage, GI bleeding, intracranial, intra-abdominal or intrathoracic bleeding, fractures

(Desired Factor VIII Increase)

A

Major

182
Q

FVIII Level Required and Frequency:
- 20 to 40
- 12 to 24 hrs

(Desired Factor VIII Increase)

A

Mild

183
Q

FVIII Level Required and Frequency:
- 30 to 60
- 12 to 24 hrs

(Desired Factor VIII Increase)

A

Moderate

184
Q

FVIII Level Required and Frequency:
- 60 to100
- 8 to 24 hrs

(Desired Factor VIII Increase)

A

Major

185
Q

Duration (Days):
At least 1 day until bleeding resolution is achieved

(Desired Factor VIII Increase)

A

Mild

186
Q

Duration (Days):
Until pain and acute disability are resolved (around 3 to 4 days)

(Desired Factor VIII Increase)

A

Moderate

187
Q

Duration (Days):
Until resolution of bleed (around 7 to 10 days)

(Desired Factor VIII Increase)

A

Major

188
Q

Type of Surgery:
Including tooth extraction

A

Minor

189
Q

Type of Surgery:
Intracranial, intraabdominal or
intrathoracic, or joint replacement
surgery

A

Major

190
Q

● The most common bleeding disorder
● It is carried on chromosome 12 and occurs equally in men and women
● A blood disorder in which the blood does not clot properly
● A genetic disorder caused by missing or defective von Willebrand factor (vWF), a clotting protein

A

von Willebrand Disease (vWD)

191
Q

● VWF binds to _________, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process

A

factor VIII

192
Q

Blood contains many proteins that help the body stop bleeding, one of these proteins is called

A

von Willebrand factor (vWF)

193
Q

● Important role in primary hemostasis
● Binds platelets
● Binds endothelial components
● Forms adhesive bridge b/w platelets and vascular subendothelial structures
● Contributes to fibrin clot formation (carries factor VIll)

A

Von Willebrand Factor (VWF)

194
Q

Treatment for vWD

A

Desmopressin (DDAVP)

195
Q

● Treatment of acquired hemophilia A
● Approved October 2014
● Recombinant

A

Obizur

195
Q

● Treatment of acquired hemophilia A
● Approved June 2014
● Factor VIII recombinant; Fe fusion protein

A

Eloctate

196
Q

● Treatment and prophylaxis of hemophilia A
● Approved September 2015
● Recombinant factor VIII

A

Nuwiq

197
Q

● Treatment of hemophilia A
● Approved May 2016
● Recombinant

A

Afstyla

197
Q

● Treatment of hemophilia A
● Approved March 2016
● Recombinant
● Use infusion line provided with product as it has in-line filter

A

Kovaltry

198
Q

● Prophylaxis and control of hemophilia B
● Approved June 2013
● Recombinant factor IX

A

Rixubis

199
Q

● Treatment of hemophilia B
● Approved March 2014
● Recombinant; Fe fusion protein

A

Alprolix

199
Q

● Treatment of hemophilia B
● Approved March 2016
● Recombinant; albumin fusion protein

A

Idelvion

3rd shift Topics (12 decks)

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