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Haematology and Immunology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

Responses in vessel wall damage

A

Primary
- platelet plug formation
- platelets bind to abnormal surface area for adherence, activation and aggregation
- vWF - bind to cells and surfaces to form an adequate platelet plug
- wall
Secondary
- fibrin plug formation

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2
Q

Mucosal pattern bleeding is caused by what?

A

Platelets problems

Von Williebrand problems

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3
Q

Presentation of coagulation factor bleeding

A 
Bleed into muscles/joints
Different patterns of bleedings into e.g. 
- knees, ankles
- shoulders
- elbows
- less so hips
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4
Q

Causes of haemorrhagic diathesis

A 
Any quantative or qualitative abnormality 
Inhibition of function of
- platelets
- vWF
- coagulation factors
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5
Q

What is haemorrhagic diathesis?

A

The tendency to bleed

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6
Q

History of bleeding - to ask about

A 
Bruises (girls have easy bruising) 
Epistaxis
Post surgical bleeding
- dental
- circumcision 
- tonsillectomy
- appendicectomy 
Menorrhagia 
- VERY relevant 
- girls who dont have menorrhagia from menarche are then very unlikely to have von Willebrand disease 
PPH
Post trauma 
FH
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7
Q

Platelet type bleeding will cause….

A 
Mucosal bleeding
Epistaxis (both nostrils)
Purpura
Menorrhagia 
GI bleeding
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8
Q

Coagulation type bleeding will cause….

A

Articular - knees, ankles, shoulders
Muscle haematoma
CNS
muscle wasting

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9
Q

What questions to ask to figure out if it is a congenital or acquired bleeding disorder?

A

Previous episodes
age of first event
previous surgical challenges
associated history

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10
Q

Which factor is affected in haemophilia type A?

A

factor 8

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11
Q

Which factor is affected in haemophilia type B?

A

Factor 9

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12
Q

Genetics of haemophilia

A

X linked

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13
Q

In haemophilia, what does the severity of bleeding depend on?

A

The residual coagulation factor activity

  • <1% = severe
  • 1-5% moderate = do not bleed spontaneously
  • 5-30% = precautions to be put in place for surgery etc so do not come to too much harm
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14
Q

Presentation of haemophilia

A 
Hemarthrosis 
- hinge and weight bearing joints 
Muscle haematoma 
- calves, biceps particularly
CNS bleeding (less common)
Retroperitoneal bleeding (less common) 
Post surgical bleeding 
- preventable with clinical management
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15
Q

Complications of haemophilia

A

Synovitis
- can result in loss of articular cartilage due to macrophages in the blood producing an inflammatory response
Chronic haemophilic arthropathy
Neurovascular compression (compartment syndromes)
Other sequelae of bleeding (stroke)

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16
Q

Diagnosis of haemophilia

A 
Clinical 
- present between 6 months - 2 y/o 
- baby walking then stops walking (due to bleeding into joints)
Screening tests for coagulation 
- prolonged APTT 
- normal PT 
Reduced FVIII or FIX 
Genetic analysis
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17
Q

Treatment of bleeding diathesis

A

Coagulation factor replacement FVIII/IX (aim to keep trop > 2%)
DDAVP
tranexamic acid
Emphasis on prophylaxis in severe haemophilia
- 1 every 2 weeks or twice a week for Haemophilia A
Gene therapy

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18
Q

Haemophilia treatment

A 
Splints
physio 
analgesia
synovectomy 
joint replacement
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19
Q

Haemophilia complications of treatment

A

Viral infection (HIV, HBV etc)
Development of inhibitors
- Anti FVIII Ab (if severe and have no FVIII antigen, will form a response to the coagulation factor replacement)
- rare in FIX as fewer null mutations
DDAVP
- desmopressin (factor 8 and 9 go up for a day which allows you to have surgery etc)

20
Q

What does desmopressin/DDAVP do?

A

Promotes the release of von Willebrand factor

21
Q

What is DDAVP/Desmopressin contraindicated in?

A 
Elderly 
High risk of vascular disease
Children < 3 y/o 
due to 
- MI 
- hyponatraemia (babies)
22
Q

How common is von Willebrand disease?

A

Common - 1 in 200

23
Q

Presentation of von Willebrand disease

A

Platelet type bleeding (mucosal)

24
Q

Pathology of Von Willebrand disease, either ….

A

Quantative (dont make enough)

Qualitive (doesn’t function right)

25
Q

Types of Von Willebrand disease

A 
Type 1 
- quantative deficiency 
Type 2 (A, B, M, N)
- qualitative deficiency determined by the site of the mutation in relation to vWF function 
Type 3
- severe (complete deficiency)
26
Q

Treatment of von Willebrand disease

A

vWF concentrate / factor VIII concentrate or
DDAVP
Tranexamic acid
OCP

27
Q

Causes of acquired bleeding disorders

A 
Thrombocytopenia 
Liver failure
Renal failure 
DIC
Drugs (the big group of disorders, most common causing bleeding) 
- warfarin
- heparin 
- aspirin 
- clopidogrel 
- rivaroxaban 
- apixaban 
- dabigatran
28
Q

What is thrombocytopenia?

A

Abnormally low levels of thrombocytes/platelets in blood

29
Q

Causes of thrombocytopenia

A

Decreased production
- marrow failure (anaemia and thrombocytopenia symptoms)
- aplasia
- infiltration
Increased consumption
- Immune
- most common - ITP
- non immune DIC
- inappropriate activation of cascade (using up the platelets and coagulation factors)
- burns, sepsis, obstetric emergencies
Hypersplenism
- massive spleen = platelets get stuck in it
- very rarely have a low platelet count (<40)

30
Q

Presentation of thrombocytopenia

A 
Petechiae 
- mucosal more dangerous
ecchymosis 
mucosal bleeding
rare CNS bleeding
31
Q

What level of platelet counts are rarely associated with bleeding disorders?

A

> 40

32
Q

What does ITP stand for?

A

Idiopathic thrombocytopenic purpura

33
Q

What is ITP?

A

A disorder that can lead to easy or excessive bleeding / bruising - the bleeding resulting from abnormally low levels of platelets

34
Q

Associations of ITP

A

Infection (EBC, HIV, glandular fever)
Collagenosis
lymphoma
drug induced (quinine most common)

35
Q

Treatment of ITP

A

Steroids
IV IgG
Splenectomy
Thrombopoietin analogues (eltrombopag and romiplostim)

36
Q

Pathology of liver failure in respect to bleeding

A

Factor I, II, V, VII, VIII, IX, X, XI
Prolonged PT
APTT reduced fibrinogen

37
Q

Treatment of liver failure affecting bleeding

A

Cholestasis Vit K dept factor deficiency
Factor II, VII, IX, X
Replacement FFP
Vitamin K

38
Q

In liver failure, where does most bleeding occur?

A

From structural lesions - varices

39
Q

Pathology of haemorrhagic disease of the newborn

A

Immature coagulation systems
Vitamin K deficient diet (esp breast)
Fatal and incapacitating haemorrhage

40
Q

How is haemorrhagic disease of the newborn completely prevented?

A

1mg IM administration of Vit K at birth

41
Q

How does tranexamic acid work?

A

Reversibly binds to lysine receptor sites on plasminogen or plasmin, preventing plasmin from binding to and degrading fibrin

42
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand disease

43
Q

Inheritance of VWD

A

Autosomal dominant

44
Q

Type 3 VWD is what type of inheritance?

A

Autosomal recessive

45
Q

What % of patients have type I VWD?

A

80%

46
Q

What is the most common cause of isolated thrombocytopenia?

A

ITP

47
Q

What would make you think of haemophilia A/B?

A

Hemarthrosis without trauma

Haematology and Immunology (18 decks)

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