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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Name 4 normal haemostatic mechanisms

A

Vessel wall
vWF
Platelets
Coagulation factor

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2
Q

Name the primary haemostatic responses

A

Primary Platelet plug
Platelets
vWF
Vessel wall

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3
Q

Name the secondary haemostatic response

A

Fibrin plug formation

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4
Q

What is meant by haemorrhagic diathesis?

A

Susceptibility to bleeding

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5
Q

What causes haemorrhagic diathesis?

A

Hypocoagulability
Inhibition of function of platelets, vWF or coagulation factors

Can be quantitative or qualitative abnormality

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6
Q

5 Essential Questions in a Bleeding History

A 
Has the patient a bleeding disorder?
Severity of disorder
Pattern of bleeding
Congenital or Acquired
Mode of Inheritance
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7
Q

Particular events to enquire about in bleeding history

A 
Bruising
Epistaxis
Post-surgical bleeding
Menorrhagia
Post-partum haemorrhage
Post trauma
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8
Q

Examples of post surgical bleeding

A

Dental surgery
Circumcision
Tonsillectomy
Appendicectomy

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9
Q

2 factors to determin severity of bleeding

A

Is it spontaneous and unprovoked?

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10
Q

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

A

Platelet abnormality

Coagulation factor abnormality

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11
Q

List the 5 patterns of bleeding seen when platelets are abnormal

A 
Mucosal
Epistaxis
Purpura
GI
Menorrhagia
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12
Q

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

A

CNS
Articular
Muscular

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13
Q

Describe the presentation of severe thrombocytopenia

A 
Muscosal type bleeding
Petechiae which do not blanch
Mucosal bruising
Platelets typically less than 10
May present with extremely heavy periods
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14
Q

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

A

Coagulation factor deficiency

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15
Q

Questions to ask to determine if condition congenital or aqcuired?

A

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

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16
Q

When does severe haemophilia typically present?

A

6 months to 2 years

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17
Q

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

A

Severe haemophilia

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18
Q

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

A

Relation to family member with condition

Sex of affected family member

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19
Q

How are haemophilia A and B inherited?

A

X-linked

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20
Q

Does Haemophilia A differ from Haemophilia B in phenotype?

A

No

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21
Q

Describe the variable severity in haemophilic conditions

A

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

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22
Q

Which joints are mostly affected in haemophilia A and B?

A

Ankle
Any weight bearing joints
Hinge joints

23
Q

Name 5 clinical features of Haemophilia

A 
Haemarthrosis
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding
24
Q

Describe the symptoms of haemarthosis

A 
Tingling
Throbbing
painful
Restricts movement
Joint immobilised
25
Q

In which muscle can haematoma occur and will require extended treatment

A

Iliopsoas

26
Q

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

A

Muscle atrophy

27
Q

How can post-surgical bleeding be prevented in haemophiliacs?

A

Elevate factor viii and ix for one day

28
Q

4 Clinical complications of haemophilia

A

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

29
Q

Bony changes on X-ray of End stage haemophilic arthropathy

A

Cystic changes
Loss of cartilage
Reduced bone density

30
Q

How to diagnose haemophilia?

A

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

31
Q

Treatment of haemophilic diathesis

A 
Replace clotting factors
Recombinant products
DDA VP  - Desmopressin
Tranexamic acid
Emphasise Prophylaxis in severe cases
Gene therapy
Subcutaneous emicizumab injection
32
Q

Other management in haemophilia

A 
Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement
33
Q

Treatment complications

A

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

34
Q

Inheritance of VonWillebrand Disease

A

Autosomal dominant

35
Q

Which type of bleeding is seen in Von Willebrand disease?

A

Platelet type - mucosal

36
Q

How many types of vonWillebrand deficiency are there?

A

3

37
Q

Is Type 1 VonWillebrand Disease a qualitative or quantitative deficiency?

A

Quantitative

38
Q

Is Type 2 VonWillebrand Disease a qualitative or quantitative deficiency?

A

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

39
Q

Is Type 3 VonWillebrand Disease a qualitative or quantitative deficiency?

A

Type 3 is a COMPLETE deficiency - severe

40
Q

How is vW disease treated?

A

vWF concentrate or DDA VP
Tranexamic acid
Topical application
OCP

41
Q

Name 5 types of acquired bleeding disorders

A 
Thrombocytopenia
Liver failure
Renal failure
DIC
Drugs
42
Q

Which drug use may result in an acquired bleeding disorder

A

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

43
Q

2 ways in which thrombocytopenia occurs

A

Increased consumption

Decreased production

44
Q

3 causes of decreased platelet production

A

Marrow failure
Infiltration
Aplasia

45
Q

3 causes of increased platelet consumption

A

Immune ITP
Non immune DIC
Hypersplenism

46
Q

4 Clinical features of thrombocytopenia

A

Petechiae
Ecchymosis
Mucosal Bleeding
Rarely CNS bleeding

47
Q

4 conditions/events associated with Immune Thrombocytopenic Purpura

A

Infection - EBV, HIV
Collagenosis
Lymphoma
Drug-induced

48
Q

Treatment of ITP

A

Steroids
IV IgG
Splenectomy
Thrombopoietin analogues

49
Q

Examples of thrombpoietin analgoues

A

Eltrombopag

Romiplostim

50
Q

How might liver failure cause a bleeding disorder?

A

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

51
Q

Treatment of bleeding disorder in case of liver failure

A

Vitamin K

Replacement FFP

52
Q

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

A

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

53
Q

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

A

Retroperitoneal and intracranial

54
Q

How is Haemolytic Disease of the Newborn preventable at birth?

A

Administration of Vitamin K

Haematology (7 decks)

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