Heritable Bleeding Disorders Flashcards Preview

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Flashcards in Heritable Bleeding Disorders Deck (28):
1

The balance between bleeding and clotting

Haemostatic balance

2

What 2 mechanisms for the haemostatic plug?

Aggregation and coagulation

3

3 main classes of bleeding disorders

Platelet dysfunction
Vessel wall
Coagulation defect

4

Which classes of bleeding disorders results in bleeding in the skin or mucosa

Platelet dysfunction
Vessel wall

5

Which classes of bleeding disorders result in deep muscular joint bleeds following trauma?

Coagulation defect

6

What drug affects the normal function of platelets

Aspirin

7

What 2 diseases affect the vessel wall

Ehler's danlos
Scurvy

8

What disease is the abnormal interaction between platelets and the vessel wall

Von Willebrand Disease

9

4 characteristics of bleeding disorders due to vascular/platelet defects

Petechiae and superficial bruises
Skin/mucous membranes
Spontaneous
Prolonged and non recurrents

10

4 characteristics of bleeding disorders due to a defect in coagulation

Deep spreading haematoma
Haemarthrosis
Retroperitoneal bleeding
Prolonged, often recurrent

11

Red spots on the skin that do not blanch with pressure and are not palpable

Petechiae

12

Is von willebrand disease AR or AD

AD

13

What is the function of von willebrand factor?

Helps platelets adhere to vessel wall. Acts as a carrier for factor 8

14

Most common heritable bleeding disorder

Von Willebrand disease

15

Symptoms of von willebrand disease

Mucocutaneous bleeding including menorrhagia (heavy periods)
Post operative/post partem bleeding

16

If you are blood group O are you more or less likely to have von willebrand disease?

Less

17

Treatment for von willebrand disease

Antifibrinolytics
Factor concentrations (including VWF)
DDAVP-desmopressin-releases VWF from stores inside endothelial cells

18

What is the most common of coagulation factor deficiencies?

Haemophilia

19

Activated partial thromboplastin time measures which pathway and up until what factor?

Intrinsic
Thrombin

20

Prothrombin time measures which pathway and up until what factor?

Extrinsic
Thrombin

21

The thrombin clotting time measures what part of the clotting cascade

Thrombin>Fibrinogen>fibrin

22

The euglobulin lysis time measures what part of the clotting cascade

Plasmin>fibrin degredation products

23

What inheritence pattern are the haemophilias?

X linked recessive

24

Haemophilia A is a deficiency in which factor

8

25

Haemophilia B is a deficiency in which factor

9

26

Which is more common, haemophilia A or B?

A

27

Is severity variable or constant between family members with haemophilia?

Consistnet

28

Treatment for haemophilia

Antifibrinolytics
Replace the missing clotting factor-prophylaxis
DDAVP-desmopression that releases VWF stores from inside endothelial cells