Bleeding disorders Flashcards

1
Q

What are the 3 reasons for abnormal bleeding?

A

Vascular disorder, Thrombocytopenia (defective platelet function) and defective coagulation

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2
Q

In coagulation disorders, where does bleeding occur?

A

Into the joints or soft tissue

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3
Q

In vascular and platelet disorders, where does bleeding take place?

A

From the mucous membranes and skin

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4
Q

What are the genetic probability for people inheriting bleeding disorders?

A

In disorders affecting platelets, it is equal in both sexes, while coagulation diseases are X-linked and have occurred in males >80% of the time

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5
Q

What is thrombocytopenia?

A

The most common platelet disorder and most commonly acquired. Can be due to a failure of production or increased destruction. It is characterised by spontaneous skin purpura, mucosal haemorrhage and prolonged bleeding after trauma

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6
Q

What is platelet function analysis (PFA 100)?

A

An alternative to bleeding time that has a quick and easy determination of platelet function. Citrated whole blood is aspirated at high shear rates through disposable cartridges containing an aperture coated with either collagen and epinephrine or collagen and ADP

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7
Q

What can be used to prevent atherothrombosis?

A

Aspirin as it permanently inactivates cyclooxygenase

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8
Q

What are the most common acquired bleeding disorder?

A

Vitamin K deficiency, liver disease, disseminated intravascular coagulation, massive blood loss, auto-antibodies

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9
Q

What are the most common inherited bleeding disorders?

A

Hemophilia, Von Willebrand disease, Bernard-Soulier syndrome, Glanzmann’s thrombasthenia

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10
Q

What is our main source of vitamin K and what does it do?

A

Dark green veg and serves as an essential co-factor for carboxylase that catalyses carboxylation of glutamic acid residues on vitamin K-dependent proteins

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11
Q

What are key vitamin K-dependant proteins?

A

Coagulation proteins - factor 2, 7, 9 and 10

Anticoagulation proteins - protein C, S and Z (all require carboxylation)

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12
Q

What happens when glutamic has been carboxylated by vitamin K?

A

Can bind calcium more effectively

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13
Q

What can cause vitamin K deficiency?

A

Malabsorption of fat-soluble vitamins, oral anticoagulation therapy (warfarin), liver disease, dietary deficiency associated with antibiotic treatment that destroys gut bacteria that can synthesise Vit K

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14
Q

What clotting factors are produced by the liver?

A

1, 2, 5, 7, 9 10 and some 8

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15
Q

What is disseminated intravascular coagulation (DIC)?

A

Inappropriate activation of blood coagulation, causing a generation and deposition of fibrin leading to microvascular thrombi in various organs. Disease can be fatal

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16
Q

What will be found in lab tests for someone with DIC?

A

Low platelet count, low fibrinogen concentration, high levels of fibrin degradation production (D-Dimers), thrombin time, PT and activated partial thromboplastin time (APTT) prolonged

17
Q

What is defined as massive blood loss?

A

50% of blood vol loss within 3 hours or a rate of loss of 150ml/hour

18
Q

Describe haemophilia

A

Defined as a love of blood, causes prolonged and excessive bleeding. Is X-chromosome linked, haemophilia A is the deficiency of factor 8, while haemophilia B is the deficiency of factor 9 (Christmas disease). Haemophilia A is the most common of the severe inherited bleeding disorders affecting 1:5000 male births

19
Q

How can you distinguish between haemophilia A and VWD?

A

Haemophilia A - normal VWF and low F8

VWD - low VWF and low F8

20
Q

What are the therapy options for the different severities of haemophilia?

A

Severe - concentrated factors usually prophylactically to prevent bleeds
Moderate - concentrated factors when needed or desmopressin, which induces a short-term rise in factor 8 and VWF
Mild - desmopressin when compromised

21
Q

What are the different types of VWD?

A

Type 1 - partial quantitive VWF deficiency
Type 2 - qualitative VWF deficiency
Type 3 - total VWF deficiency - platelet transfusions may be needed