Inherited and acquired bleeding disorders (haematology) Flashcards

1
Q

What causes bleeding disorders? (4)

A

Vascular disorders
Platelets disorders
Von willebrand factor disorders
Defective coagulation

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2
Q

What do …. deficiency mean?
1. Quantitative
2. Qualitative

A
  1. Not enough platelets, clotting factors etc.
  2. There are platelets, clotting factors etc but they are not working (have a defect).
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3
Q

What are the vascular bleeding disorders we have? (2)

A

•Inherited
-Hereditary haemorrhagic telangiectasia
-Connective tissue disorders
•Acquired
-Senile purpura
-Steroid purpura
-Scurvy

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4
Q

What are the causes of thrombocytopenia? (5)

A

Inherited
-Failure of platelet production.
Acquired
-Failure of platelet production.
-Sequestration
-Dilution
-Increase in platelet destruction.

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5
Q

What are the disorders of platelet function? (7)

A

•Hereditary
-Bernard-soulier syndrome
-Storage pool diseases
-Thrombasthena
•Acquired
-Antiplatelet drugs
-Hyperglobulinaemia
-Uraemia
-Myeloproliferative amd myelodysplastic disorders.

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6
Q

What are the hereditary coagulation disorders we have? (4)

A

-Factor VIII deficiency (haemophilia A)
-Factor IX deficiency (haemophilia b)
-Von willebrand disease
-Deficiencies of other coagulation factors.

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7
Q

What are the acquired coagulation disorders? (5)

A

-Massive transfusion syndrome
-Deficiency of Vit-K dependent clotting factors (2,7,9,10)
-Liver disease
-Disseminated intravascular coagulation.
-Coagulation caused by antibodies.

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8
Q

What is the diagnostic approach towards bleeding disorders? (4)

A

-Take family history.
-Physical examination
-Laboratory screening
-Confirmatory testing

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9
Q

Why is calcium removed when the blood lab test is done?

A

To prevent clotting of the blood.

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10
Q

What is Prothrombin Time (PT) ?

A

It is the time required for fibrin clot to form after the addition of thromboplastin to citrated platelet poor plasma.

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11
Q

What is the normal range for PT?

A

10-14 seconds

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12
Q

What does the PT test results indicate when they are:
1. Isolated prolonged
2. Shortened
3. Also aPTT is prolonged

A
  1. Factor VII deficiency
  2. Vitamin K supplementation, Fresh frozen plasma transfusion.
  3. Vitamin K deficiency, Vitamin K antagonists, liver disease, Direct thrombin inhibitors, Factor V and VIII deficiency, afibrinogenaemia or dysfibrinogenaemia.
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13
Q

What is Activated Partial Thromboplastin Time (aPTT)?

A

It is the time required for a fibrin clot to form after the addition of contact activator, phospholipid and calcium in citrated platelet poor plasma.

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14
Q

What is the normal range of aPTT?

A

25-38 seconds

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15
Q

Which clotting factors are vitamin K dependent?

A

II, VII, IX and X.

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16
Q

What does the aPTT test results indicate, when they are:
1. Shortened
2. Prolonged (3)

A
  1. Elevated factor VIII activity.
  2. Deficiency in factor II, V, VIII, IX, X and XII (any).
    Non-specific inhibitors
    Specific coagulation factor inhibitors .
17
Q

What is Thrombin Time (TT)?

A

It is the time required for a fibrin clot to form after the addition of thrombin to citrated platelet poor plasma.

18
Q

Normal range for TT?

A

15-19 seconds

19
Q

What does the TT results indicate if they are:
1. Shortened
2. Prolonged (6)

A
  1. Coagulation activation
  2. Hypoalbuminaemia
    Hypofibrogenaemia
    Increased concentration of fibrin degradation products.
    Dysfibrogenaemia
    Thrombin inhibitors
    Paraproteinaemia
20
Q

Which test should be performed after aPTT and/or PT were prolonged, and does that test help us with? (3)

A

-Perform mixing or correction assay, it helps to determine where the prolonged clotting times are due to:
-Circulating inhibitors of coagulation factors.
-Clotting factor deficiency

21
Q

How do we know whether the problem is with factor deficiency or inhibitors?

A

If the clotting time normalize, it is a factor deficiency, if it remains prolonged it is inhibitors.

22
Q

What are the confirmatory tests that are done after the mixing studies? (2)

A

-In a case of a factor deficiency, factor assay is performed to measure the specific factor activity.
-In a case of inhibitor a factor assay is done to measure the specific factor activity and inhibitor assay to measure the concentrations of the inhibitor of the deficient coagulation factor and/or lupus anticoagulant.