Bleeding Disorders

Question 1

Name 4 normal haemostatic mechanisms

Answer 1

Vessel wall
vWF
Platelets
Coagulation factor

Question 2

Name the primary haemostatic responses

Answer 2

Primary Platelet plug
Platelets
vWF
Vessel wall

Question 3

Name the secondary haemostatic response

Answer 3

Fibrin plug formation

Question 4

What is meant by haemorrhagic diathesis?

Answer 4

Susceptibility to bleeding

Question 5

What causes haemorrhagic diathesis?

Answer 5

Hypocoagulability
Inhibition of function of platelets, vWF or coagulation factors

Can be quantitative or qualitative abnormality

Question 6

5 Essential Questions in a Bleeding History

Answer 6

Has the patient a bleeding disorder?
Severity of disorder
Pattern of bleeding
Congenital or Acquired
Mode of Inheritance

Question 7

Particular events to enquire about in bleeding history

Answer 7

Bruising
Epistaxis
Post-surgical bleeding
Menorrhagia
Post-partum haemorrhage
Post trauma

Question 8

Examples of post surgical bleeding

Answer 8

Dental surgery
Circumcision
Tonsillectomy
Appendicectomy

Question 9

2 factors to determin severity of bleeding

Answer 9

Is it spontaneous and unprovoked?

Question 10

Different patterns of bleeding occur depednding on the abonormality of particular factors. Which 2 factors can we distinguish from pattern of bleeding?

Answer 10

Platelet abnormality

Coagulation factor abnormality

Question 11

List the 5 patterns of bleeding seen when platelets are abnormal

Answer 11

Mucosal
Epistaxis
Purpura
GI
Menorrhagia

Question 12

List the 3 patterns of bleeding seen when coagulation factors are affected

Answer 12

CNS
Articular
Muscular

Question 13

Describe the presentation of severe thrombocytopenia

Answer 13

Muscosal type bleeding
Petechiae which do not blanch
Mucosal bruising
Platelets typically less than 10
May present with extremely heavy periods

Question 14

Patient presents with a hot, swollen, tender knee joint and is holding joint in position. What is the likely haematological deficiency?

Answer 14

Coagulation factor deficiency

Question 15

Questions to ask to determine if condition congenital or aqcuired?

Answer 15

Previous episodes
Age at 1st event
Previous surgical challenges
Family history

Question 16

When does severe haemophilia typically present?

Answer 16

6 months to 2 years

Question 17

Little boy, stops walking, refuses to weight bear. What is a likely diagnosis?

Answer 17

Severe haemophilia

Question 18

If a condition is hereditary what 2 pieces of information are needed?

Answer 18

Relation to family member with condition

Sex of affected family member

Question 19

How are haemophilia A and B inherited?

Answer 19

X-linked

Question 20

Does Haemophilia A differ from Haemophilia B in phenotype?

Answer 20

No

Question 21

Describe the variable severity in haemophilic conditions

Answer 21

Severity depends on residual coagulation activity
Severe spontaneous bleeding
Bleeding on appropriate provocation
Mild- incidental discovery

Question 22

Which joints are mostly affected in haemophilia A and B?

Answer 22

Ankle
Any weight bearing joints
Hinge joints

Question 23

Name 5 clinical features of Haemophilia

Answer 23

Haemarthrosis
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding

Question 24

Describe the symptoms of haemarthosis

Answer 24

Tingling
Throbbing
painful
Restricts movement
Joint immobilised

Question 25

In which muscle can haematoma occur and will require extended treatment

Answer 25

Iliopsoas

Question 26

What has been observed particularly in patients Over 50 with untreated haemophilia

Answer 26

Muscle atrophy

Question 27

How can post-surgical bleeding be prevented in haemophiliacs?

Answer 27

Elevate factor viii and ix for one day

Question 28

4 Clinical complications of haemophilia

Answer 28

Synovitis Chronic Haemophilic Arthropathy Neurovascular compression (compartment syndromes) Other sequelae of bleeding - Stroke

Question 29

Bony changes on X-ray of End stage haemophilic arthropathy

Answer 29

Cystic changes Loss of cartilage Reduced bone density

Question 30

How to diagnose haemophilia?

Answer 30

Genetic analysis Chorionic villus sampling, sequence gene / take blood from cord Clinically Prolonged APTT, Normal PT, Reduced F VIII/IX

Question 31

Treatment of haemophilic diathesis

Answer 31

``` Replace clotting factors Recombinant products DDA VP - Desmopressin Tranexamic acid Emphasise Prophylaxis in severe cases Gene therapy Subcutaneous emicizumab injection ```

Question 32

Other management in haemophilia

Answer 32

``` Splints Physiotherapy Analgesia Synovectomy Joint replacement ```

Question 33

Treatment complications

Answer 33

Viral infection - HBV, HCV, HIV Inhibitors- AntiFactor VIII antibodies seen in babies; need to induce tolerance DDA VP - can cause hyponatraemia and MI in babies

Question 34

Inheritance of VonWillebrand Disease

Answer 34

Autosomal dominant

Question 35

Which type of bleeding is seen in Von Willebrand disease?

Answer 35

Platelet type - mucosal

Question 36

How many types of vonWillebrand deficiency are there?

Answer 36

3

Question 37

Is Type 1 VonWillebrand Disease a qualitative or quantitative deficiency?

Answer 37

Quantitative

Question 38

Is Type 2 VonWillebrand Disease a qualitative or quantitative deficiency?

Answer 38

Qualitative A/B/M/N Depends on site of mutation

Question 39

Is Type 3 VonWillebrand Disease a qualitative or quantitative deficiency?

Answer 39

Type 3 is a COMPLETE deficiency - severe

Question 40

How is vW disease treated?

Answer 40

vWF concentrate or DDA VP Tranexamic acid Topical application OCP

Question 41

Name 5 types of acquired bleeding disorders

Answer 41

``` Thrombocytopenia Liver failure Renal failure DIC Drugs ```

Question 42

Which drug use may result in an acquired bleeding disorder

Answer 42

Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Dabigatran, Apixiban, Bivalirudin

Question 43

2 ways in which thrombocytopenia occurs

Answer 43

Increased consumption | Decreased production

Question 44

3 causes of decreased platelet production

Answer 44

Marrow failure Infiltration Aplasia

Question 45

3 causes of increased platelet consumption

Answer 45

Immune ITP Non immune DIC Hypersplenism

Question 46

4 Clinical features of thrombocytopenia

Answer 46

Petechiae Ecchymosis Mucosal Bleeding Rarely CNS bleeding

Question 47

4 conditions/events associated with Immune Thrombocytopenic Purpura

Answer 47

Infection - EBV, HIV Collagenosis Lymphoma Drug-induced

Question 48

Treatment of ITP

Answer 48

Steroids IV IgG Splenectomy Thrombopoietin analogues

Question 49

Examples of thrombpoietin analgoues

Answer 49

Eltrombopag | Romiplostim

Question 50

How might liver failure cause a bleeding disorder?

Answer 50

Liver produces coagulation factors I, II, V, VII, VIII, IX, X, XI Bile is needed for vitamin K

Question 51

Treatment of bleeding disorder in case of liver failure

Answer 51

Vitamin K | Replacement FFP

Question 52

Haemolytic disease of the newborn occurs due to an immature coagulation system. Which clotting factors are deficient?

Answer 52

Vitamin K dependent factors - II, VII, IX and X

Question 53

HDN can cause fatal haemorrhage to occur- what sites in particular?

Answer 53

Retroperitoneal and intracranial

Question 54

How is Haemolytic Disease of the Newborn preventable at birth?

Answer 54

Administration of Vitamin K