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Clinical Pathology > Heritable bleeding disorders > Flashcards

Heritable bleeding disorders Flashcards

1
Q

What is the process by which platelets AGGREGATE to the injury site?

A
  • Damage to vascular wall
  • Platelets adhere to point of injury
  • Sticks to vWf (activates platelets) and then collagen
  • Platelet plugs holes
  • Require fibrin formation to stabilise clot
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2
Q

What intracellular processes are involved in the positive activation of more platelets?

A

Thromboxin release - constricts blood vessels and activates platelets

Alpha granules - coagulation factors, adhesion proteins, firbonolytics factors etc

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3
Q

What is the platelet receptor for fibrinogen?

A

Glycoprotein 2B3A

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4
Q

What is the platelet receptor for vWf?

A

Glycoprotein 1B9

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5
Q

How does aspirin mediate its function?

A

COX inhibitor - enzyme which synthesis thromboxin in platelet

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6
Q

What are the action of 2B3A antagonists?

A

Prevent platelet fibrinogen adhesion

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7
Q

What factors are activated by thrombin?

A

V, VIII, XI, XIII

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8
Q

What comprises the extrinsic coagulation cascade?

A

TF, VII

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9
Q

What comprises the extrinsic coagulation cascade?

A

TF, VII, X (along with factor V)

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10
Q

What comprises the intrinsic tissue cascade?

A

XII, XI, IX, VIII

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11
Q

What comprises the common pathway of the coagulation cascade?

A

X, V and prothrombin

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12
Q

What is the pathway that prothrombin time is timing?

A

Extrinsic/common pathway

TF substitute (fibroplatin) see how long it takes for fibrin to form)

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13
Q

What is the activated partial thromboplastin time counting?

A

Intrinsic pathway

foreign regents added e.g. silica based - activated factor 12

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14
Q

If prothrombin time is high, where do the deficiencies i.e.?

A

Factors in the extrinsic and common pathway

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15
Q

During activated partial thromboplastin timing, what is added to the mixture and why?

A

Calcium and phospholipids

Platelet substitute and some factors are calcium dependent

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16
Q

What effects the activated partial thromboplastin time?

A

Abnormalities in the INTRINSIC or common pathway

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17
Q

What 3 investigations that comprise a coagulation screen?

A
  • Prothrombin time
  • Activated partial thromboplastin time
  • Thrombin clotting Time
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18
Q

What is the thrombin clotting time timing?

A

Time from thrombin added to plasma to formation of fibrin clot

19
Q

What factors are involved in anticoagulation?

A

Protein C/S
Anti-thrombin III
Fibrinolytic system

20
Q

What does the fibrinolytic system comprise?

A

Activation of plasminogen to plasmin (T-PA; tissue plasmin activation factor)

Breakdown of fibrin to fibrinogen degradation products (by plasmin)

21
Q

How is this fibrinolytic system taken advantage of with an MI?

A

Use of T-PA to break down clot!

22
Q

How are bleeding disorders characterised?

A
  • Acquired
  • Congenital
  • Coagulation disorgers
  • Aggregation/adhesion disorders
23
Q

What sort of bleeding patters would you observe for secondary bleeding/coagulation defects?

A

Deep muscular and joint bleeds (haemarthitris)

Deep spreading haematomas

Retroperitoneal bleeding

Prolonged, recurrentbleeding

24
Q

What sort of patterns of bleeding would you observe with primary bleeding/platelet aggregation disorders? e.g. vWf

A

-Mucocutaneous bleeding inc heavy periods

Petechiae/superficial bleeds

  • Nose bleeds
  • Immediate, prolonged bleeds, non-recurrent
25
Q

What are the main types of defects you observe with platelet/vessel wall defects?

A

Reduced platelet number

Abnormal platelet function - e.g. production of thromboxane, dysfunctional receptors

Abnormal vessel wall e.g. collagen and survey

Abnormal interaction between platelet and vessel wall e.g. vW disease

26
Q

What do all platelet/vessel or coagulation defects all end up causing?

A

Prolonged bleeding

27
Q

What are the three types of vW disease?

A

Type I - some proteins absent but maintains multimeric structure - mild bleeding

Type 2 - right number of proteins but abnormal multimeric structure - mild bleeding

Type 3 - no vWf at all - severe bleeding

28
Q

What are the vW disease types inheritance patterns?

A

Type1/2 - autosomal dominant

Type 3 - autosomal recessive

29
Q

What is the other function for vWf?

A

Carrier protein for factor VIII

Therefore, reduced VIII level

Defects of coagulative disorders may present e.g. deep bleeding/haemoarthiritis

30
Q

For vWd are male or females more effected?

A

F=M (autosomal dominant inheritance)

31
Q

What is the treatment for vW disease/

A

Antifibrinolytics - tranexamic acid

Factor concentrates containing vWf

Blood donation with vWf

32
Q

What factor is associated with haemophilia A?

A

Factor 8

33
Q

What factor is associated with haemophilia B?

A

Factor 9

34
Q

What is the inheritance pattern for haemophilia A/B

A

Sex linked recessive

35
Q

What factor is von villibrand factor associated with?

A

Factor 8 - transport protein

36
Q

What is the inheritance patterns for factors I, II, V, VII, X, XIII

A

Autosomal recessive

37
Q

What is the inheritance pattern for factors XII and XI?

A

Autosomal dominant

38
Q

What are the chances of a son/daughter being a carrier/suffer for Haemophilia A/B if mother is carrier?

A

50% for son to be suffers, females being carrier

39
Q

What are the chances of the sons being suffers and daughters being carriers if father has haemophilia A/B

A

All Daughters will be carriers

No sons will be suffers

40
Q

What is the degree of haemophilia dependent on?

A

Family severity - sons will not be more/less effected - the same

41
Q

What are the degrees of severity of haemophilia?

A

Normal 50-150%
Mild 6- 50%
Moderate 1 -5%
Severe

42
Q

What are the types of bleeds you get with haemophilia? What are the types of bleeds dependent on?

A
  • Spontaneous
  • Traumatic bleeds

Severity of haemophilia - i.e. more severe, easily bleeding!

Haemoarthiritis
Muscle bleeds
Soft tissue bleeds
Life threatening e.g. cranial/airway

43
Q

What are the treatment options for haemophilia A/B

A

Replacement of clotting factors

Antifibrinolytics e.g. tranexamic acid

Clinical Pathology (50 decks)

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