Coagulopathies 9-16-14 Flashcards

1
Q

What are the 3 phases in hemostasis?

A

Vascular (vasoconstriction), platelet (plug), and plasma (coagulation)

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2
Q

What is the average life span of platelets?

A

5-9 days

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3
Q

*Define platelets (look up definition)

A

Small, disc shaped cell fragments that assist in blood clotting

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4
Q

*Define von Willebrand factor (vWE)

A

A plasma protein that is important in the platelet phase, especially adhesion and aggregation
(congenital is caused by mutations in the vWF gene)

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5
Q

*A patient presents with mucocutaneous bleeding in gums. What disorder is responsible?

A

Primary hemostasis (platelets and VW)

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6
Q

*A patient presents with abnormal bleeding into their joint spaces. What disorder is responsible?

A

Defects in coagulation factors

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7
Q

?Describe the steps involved in bleeding and clotting (recheck)

A
  • Platelet phase
  • Platelets
  • Platelet adhesion
  • Platelet activation
  • Platelet aggregation
  • Primary hemostatic platelet plug
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8
Q

a) What is the most common inherited bleeding disorder?

b) Who is affected

A

a) von Willebrand disease

b) Males and Females equally

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9
Q

It a patient has a defect in vWF, what also might be affected?

A

Factor 8

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10
Q

What type of vWF is autosomal dominant?

A

Type 1

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11
Q

What type of vWF is a result of complete absence of vW?

A

Type 3

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12
Q

What type of vWF is a result of qualitative defect of vWF?

A

Type 2

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13
Q

*What 3 tests are used to screen for vWD?

A
  • Plasma von Willebrand factor antigen
  • Plasma VWF activity
  • Factor VIII activity
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14
Q

What is the treatment for vWF type 1 and some type 2?

A
  • Stimate nasal spray

- Synthetic hormone

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15
Q

What is the treatment for vWF type 3 or type 1 trauma patients?

A

Humate P

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16
Q

Describe hemophilia

A

Sex linked inherited deficiency of Factor VIII (A) or Factor IX(B)

17
Q

What are the laboratory findings that are used to diagnose a patient with hemophilia A, B?

A

NL platelet count
Nl PT
Prolonged aPTT

18
Q

What lab test is used to diagnose Hemophilia?

A

Specific factor assays (VIII, IX)

19
Q

What treatment is used for hemophilia?

A
  • Factor replacement, pain control, rest or immobilize joint
  • Prophylaxis/on demand
  • Home therapy
20
Q
*Formulate a differential diagnosis for a bleeding disorder
(check with class notes)
A
  • primary hemostasis (PLT number or function or vW disease)

- secondary hemostasis (Hemophilia, liver, drugs, vitamin K )

21
Q

What are the 3 main causes of thrombosis (Virchow’s triad)?

A
  • Reduced blood flow (stasis)
  • Blood hypercoagulability
  • Vascular wall abnormalities
22
Q
  • Define thrombolysis
A

Lysis of blood clots by pharmacological means

23
Q

*Define thrombosis

A

Local coagulation or clotting of the blood in the veins or arteries

24
Q

What is the difference between arterial and venous thrombosis?

A
  • Venous thrombosis- coagulation system abnormalities (DVT)

- Arterial thrombosis- platelets play the predominant role (HA)

25
Q

*Describe Factor V Leiden (FVL) disorder

A

-Activated protein C inhibitor of the coagulation system that that reduces the amount of factor 5 and 8

26
Q

What is the most common inherited throbophilia?

A

Factor V Leiden(FVL) disorder

27
Q

How do you diagnose for FVL?

A

Genetic testing

28
Q

How long must a patient be off of coumadin to test for C & S deficiency?

A

2-3 weeks before testing

29
Q

What is nephrotic syndrome?

A

Loss of natural anticoagulants in urine

30
Q

*Discuss the role of malignancy in hypercoagulation

A
  • 20% of all VTEs occur in cancer

- 10% of all unprovoked VTEs will be diagnosed with cancer the year after diagnosis

31
Q

*Discuss the role of contraceptive pills (OCP) in hypercoagulation

A
  • Estrogen OCPs increase risk 3-5-fold
  • Absolute risk is 1 in 3000/year
  • Risk is further incr. by a # of risk factors (obesity, smoking, family history of VTE, presence of thrombophilias)
  • Obesity multiplies risk to 20-30 fold, or 1 in 500/yr
32
Q

*Discuss the role of Nephrotic syndrome in hypercoagulation

A
  • Loss of natural anticoagulants
  • Multiple other abnormalities (increased PLT aggregation, impaired fibrinolysis etc)
  • Increased risk of thrombosis
33
Q

*Discuss the role of Pregnancy in hypercoagulation

A
  • 4-fold increase during pregnancy & post-partum
  • 5-fold increase postpartum vs pregnancy
  • Stasis, hypercoagulability (VII,VIII,X,Fibrinogen, vWF increase, decrease in protein S)
  • Usually prophylaxis in high risk thrombophilia