3: Bleeding Disorders

Question 1

What is a bleeding disorder

Answer 1

abnormal Haemostasis that can result in diathesis

Question 2

What coagulation pathway does PT look at and how is that remembered

Answer 2

Extrinsic

Personal trainers focus on extrinsic appearance

Question 3

What factors comprise the extrinsic pathway

Answer 3

TF, 7, 10, 5, 2, Fibrinogen

Question 4

What disorders can cause only PT to be raised

Answer 4

• Heparin

- Von Wille Brand Disease

Question 5

What coagulation pathway does the APTT look at

Answer 5

intrinsic

Question 6

What factors make up the intrinsic pathway

Answer 6

12
11
10
9
8
5
2

Question 7

What 3 disorders cause raised isolated APTT

Answer 7

• Haemophilia A
• Haemophilia B
• Antiphospholipid syndrome

Question 8

What causes raised APTT and PT

Answer 8

DIC
Liver disease
Vitamin K deficiency

Question 9

What factors are vitamin K dependent

Answer 9

1972

Question 10

What is the inheritance pattern of haemophilia A

Answer 10

X-Linked recessive

Question 11

What factor does haemophilia A affect

Answer 11

F8

Question 12

Is haemophilia A or B more common

Answer 12

Haemophilia A (80%)

Question 13

Explain presentation of haemophilia A

Answer 13

• Haemoarthrosis (bleeding into joints)
• Bleeding into muscle
• Bleeding into soft tissue

Question 14

What does petechial bleeding indicate

Answer 14

Platelet deficiency - not clotting factor

Question 15

What will be seen on coagulation studies in haemophilia A

Answer 15

Prolonged APTT

Question 16

What is diagnostic of haemophilia A

Answer 16

Factor 8 assay

Question 17

What should be avoided in patients with haemophilia A and why

Answer 17

IM injections and NSAIDs - can lead to proloneed bleeding

Question 18

If a patient has mild haemophilia A what is given

Answer 18

Control bleeding with pressure and elevation

Desmopressin can increase F8

Question 19

what drug is given in mild haemophilia A to increase F8

Answer 19

Desmopressin

Question 20

what is given for major bleeds

Answer 20

Recombinant F8 transfusion

Question 21

what is haemophilia B also called

Answer 21

Christmas disease

Question 22

what is the inheritance pattern of haemophilia B

Answer 22

X-linked recessive

Question 23

what factor is deficient in haemophilia B

Answer 23

F9

Question 24

what is more common haemophilia A or B

Answer 24

Haemophilia A (90%)

Question 25

how does haemophilia B present

Answer 25

• bleeding in JOINTS, MUSCLE AND SOFT-TISSUE

- may present with epistaxis or excessive bleeding post-procedure

Question 26

what will be seen on coagulation studies in haemophilia B

Answer 26

Prolonged APTT

Question 27

what is diagnostic of haemophilia B

Answer 27

F9 assay

Question 28

how is haemophilia B managed

Answer 28

recombinant factor 9

Question 29

what is the most common inherited bleeding disorder

Answer 29

Von Wille Brand Disease

Question 30

what is the role of VWF

Answer 30

When tissue is damaged it exposes VWF which causes platelet aggregation. VWF also stabilises F8 in the coagulation cascade.

Question 31

Where is VWF found

Answer 31

Weibel-Palade bodies of endothelial cells

Question 32

What is von wille brand disease

Answer 32

deficiency of VWF

Question 33

What is the inheritance pattern of von wille brand disease

Answer 33

Autosomal dominant

Question 34

What does VWD lead to

Answer 34

deficiency of VWF and F8

Question 35

Explain symptoms of VWD

Answer 35

behaves as a platelet disorder causing epistaxis and menorrhagia.

opposed to symptoms of haemoarthrosis and bleeding into joints which are very rare

Question 36

What investigation is ordered in VWD

Answer 36

• Coagulation studies

- F8 assay

Question 37

What will be seen on coagulation studies

Answer 37

Prolonged APTT and PT

Question 38

How is bleeding in von wille brand disease managed

Answer 38

Tranexamic acid

Question 39

What medication can be given in von wille brand disease

Answer 39

Desmopressin - to increase VWF