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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What are the normal haemostatic mechanisms?

A

Vessel wall
Platelets
Von Willebrand factor
Coagulation factors

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2
Q

What is the normal haemostatic response?

A

Primary - platelet plug formation, platelets adhering to abnormal endothelium, become activated and aggregated, rely on vWF

Secondary - eventual fibrin plug formation

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3
Q

Haemorrhagic diathesis occurs due to any qualitative or quantitative abnormality or inhibition of function in what?

A

Platelets
vWF
Coagulation factors

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4
Q

What are the points you want to establish from a bleeding history?

A

Has the patient actually got a bleeding disorder?
How severe is the disorder? Context of other illness, trauma, severity etc.
Pattern of bleeding
Congenital or acquired
Mode of inheritance

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5
Q

What do you want to obtain from the history of bleeding?

A

Bruising
Epistaxis
Post-surgical bleeding - ask about previous surgery or trauma, may need to ask about specific common procedures as patients might not consider them as surgeries
Menorrhagia - has it been present from menarche, common in women with vWF mutation
Post-partum haemorrhage
Post-trauma

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6
Q

What do you want to find out about the severity of bleeding?

A

How appropriate is the bleeding i.e. is the severity of bleeding consistent with the severity of injury
Completely unprovoked bleeding vs bleeding with obvious cause
Completely unprovoked typically presents at an early age

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7
Q

What do you want to find out about the pattern of bleeding?

A

Platelet type, thrombocytopenia

  • mucosal
  • epistaxis
  • purpura
  • menorrhagia
  • GI
  • post-surgical bleeding

Coagulation factor

  • articular
  • muscle haematoma
  • CNS
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8
Q

What would you ask to distinguish between congenital or acquired bleeding?

A

Previous episodes
Age at first event, congenital will present at early age compared to a later date of acquired
Previous surgical challenges
Associated history

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9
Q

What would you ask to determine if the cause of bleeding was a hereditary disorder?

A

Family members with similar history
Sex of affected individuals - determines kind of inheritance
X-linked - carrier women and affected males
Autosomal dominant - either sex affected

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10
Q

What is the inheritance of haemophilia A and B?

A

X-linked

Identical phenotypes

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11
Q

What is the incidence of haemophilia type A and B?

A

1/10,000 type A

1/60,000 type B

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12
Q

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

A

The residual coagulation factor activity

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13
Q

What percentage of haemophilia is severe, moderate and mild?

A

< 1% severe
1-5% moderate
5-30% mild

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14
Q

Factor VIII and IX genes are on what chromosome?

A

X

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15
Q

What is the aim for treatment of haemophilia?

A

To keep factor VIII in moderate levels, to improve quality of life, life-expectancy and reduce spontaneous bleeding

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16
Q

What are the clinical features of haemophilia?

A

Haemarthrosis - hinge joints most affected, knees and ankles more severely affected than hips
Muscle haematoma - typically in large muscles, especially iliopsoas
CNS bleeding
Retroperitoneal bleeding
Post-surgical bleeding

17
Q

What are the clinical complications of haemophilia?

A

Synovitis
Chronic haemophilic arthropathy
Neurovascular compression
Other sequelae of bleeding

18
Q

How do you diagnose haemophilia?

A

Clinical presentation e.g. spontaneous bleed into a joint, paediatric

Screening blood tests

  • prolonged APTT
  • normal PT
  • normal bleeding time; detect coagulation deficiencies in different parts of coagulation cascade

Reduced FVIII or FIX depending on which is deficient

Genetic analysis - can help with management and in future pregnancies of carrier individuals

19
Q

What is the treatment of bleeding diathesis in haemophilia?

A

Previously always coagulation factor replacement
Now almost entirely done using recombinant products - made in cell lines
DDAVP - releases stored vWF in endothelial cell into the circulation, useful for those who need to have a procedure
Tranexamic acid - also beneficial in major trauma and PPH
Emphasis on prophylaxis in severe haemophilia - converting severe haemophilia to phenotype of moderate haemophilia using recombinant products
Gene therapy

20
Q

What is the treatment of acute bleeds in haemophilia?

A 
Splints
Physiotherapy 
Analgesia 
Synovectomy 
Joint replacement
21
Q

What are the complications of haemophilia treatment?

A

Viral infection

  • HIV
  • HBV, HCV
  • vCJD

Development of inhibitors

DDAVP

  • MI
  • hyponatraemia
22
Q

What are the features of von Willebrand disease?

A 
Commonest bleeding disorder
Incidence 1/200
Variable severity - majority are mild 
Autosomal inheritance
Platelet type bleeding (mucosal) 
Quantitative and qualitative abnormalities of vWF
23
Q

What type of deficiency is in von Willebrand disease type 1?

A

Quantitative deficiency - reduction in normal vWF

24
Q

What type of deficiency is in von Willebrand disease type 2?

A

Qualitative deficiency - determined by the site of mutation in relation to vWF function i.e. dependent on part of molecule that is non-functional

25
Q

What type of deficiency is in von Willebrand disease type 3?

A

Severe (complete) deficiency of vWF

26
Q

What is the treatment of von Willebrand disease?

A

vWF concentrate or DDAVP
Tranexamic acid
Topical applications
OCP

27
Q

What are the causes of acquired bleeding disorders?

A 
Thrombocytopenia 
Liver failure 
Renal failure 
DIC 
Drugs 
- warfarin 
- heparin 
- aspirin 
- clopidogrel
28
Q

What are the causes of thrombocytopenia?

A

Decreased production

  • marrow failure, inadequate production or adequate production but cells being consumed too quickly
  • aplasia
  • infiltration

Increased consumption

  • immune ITP
  • non-immune DIC
  • hypersplenism
29
Q

What are the clinical features of thrombocytopenia?

A

Petechiae
Ecchymosis
Mucosal bleeding
Rare - CNS bleeding

30
Q

What is idiopathic thrombocytopenic purpura associated with?

A

Infection, especially EBV and HIV
Collagenosis
Lymphoma
Drug induced

31
Q

What kind of condition is idiopathic thrombocytopenic purpura?

A

Immune-mediated condition

32
Q

What are the features of haemorrhagic disease of the newborn?

A

Immature coagulation systems
Vitamin K deficient - short on vitamin K dependent factors
Fatal and incapacitating haemorrhage
Completely preventable by administration of vitamin K at birth

Haematology (11 decks)

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