Bleeding Disorders Flashcards

1
Q

What are involved in the Normal Haemostatic Mechanisms?

A
  • Vessel Wall
  • Platelets
  • Von Willebrand Factor
  • Coagulation Factors
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2
Q

What is involved in the Primary and Secondary Haemostatic Response?

A

Primary;
- Platelet Plug Formation
- Platelets, vWF, Wall

Secondary;
- Fibrin Plug Formation

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3
Q

What is important to assess when faces with Haemorrhagic Diathesis (Bleeding tendencies)

A

Any quantitative or qualitative abnormality
Inhibition of function

  • Platelets
  • vWF
  • Coagulation factors

**Sometimes accompany hepatic failure. Impaired synthesis of clotting factors, reduced clearance of the products of the clotting process, and metabolic abnormalities affecting platelet function can affect normal clotting, individually or in combination.

REVIEW!

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4
Q

What should you establish in a Bleeding History?

A
  • Has the patient actually got a bleeding disorder
  • How severe is the disorder?
  • Pattern of Bleeding
  • Congenital or Acquired
  • Mode of inheritance
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5
Q

What else would you ask in the History of Bleeding?

A
  • Bruising (Mucosal, easy bleeding?)
  • Epistaxis

Response to challenges
- Post-surgical bleeding
- Dental extractions

  • Menorrhagia (Start when period started or later in life (different cause))
  • Post-partum haemorrhage (Surgery or Dentist?)
  • Post-trauma
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6
Q

What tool do we use to help diagnose Bleeding severity?

A

ISTH Bleeding Assessment Tool Score

Used to diagnose how significant the bleeding is, the severity and if it’s appropriate for the trauma caused

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7
Q

What should you ask about in the “Pattern Of Bleeding” History?

A

Platelet type:
Mucosal Bleeding? (More in line with primary, haemostatic, von willebands)
- Epistaxis
- Purpura
- Menorrhagia
- GI

Coagulation Factor:
Articular?
- Muscle Haematoma
- CNS

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8
Q

What is this? INSERT PICTURE

A

Bruising in petechial rash - low platelets. Need urgent platelet count, will be low

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9
Q

What is this? (INSERT PIC)

A

Knee haemarthrosis, knee haemophilia, bled into knee

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10
Q

What is this? (INSERT PIC)

A

Rectus Sheath Haematoma - Seen in warfarin and if over anti-coagulated or haemophilia

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11
Q

What is this? (INSERT PIC)

A

Intracranial Haemorrhage in Haemophilia - Very rare now with good care

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12
Q

What do you want to ask in “Congenital or Acquired?”

A
  • Previous Episodes ?
  • Age at first event
  • Previous surgical challenges
  • Associated History
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13
Q

If you think the disorder is Hereditary what should you establish?

A
  • Family members with similar history
  • Sex (or inheritance patterns)
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14
Q

What kind of inheritance is this? INSERT PIC

A

Autosomal dominant (von Willebrand’s)

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15
Q

What are the features of haemophilia A and B?

A
  • X-Iinked
  • Identical phenotypes
  • 1 in 10,000 (A) and 1 in 60,000 (B)
  • Severity of bleeding depends on the residual coagulation factor activity
  • <1% Severe (Have to have very low factor level for this)
  • 1-5% Moderate
  • 5-30% Mild (Start to develop symptoms when 1 year old)
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16
Q

What Haemopholia is more common?

A

Haemophilia A 1 in 10,000 (B is 1 in 60,000)

17
Q

What are the Clinical features of haemophilia A and B?

A
  • Haemarthrosis
  • Muscle haematoma
  • CNS bleeding (disappearing nowadays)
  • Retroperitoneal bleeding
  • Post surgical bleeding
18
Q

What is Haemophilia?

A

Coagulation factor defect so more bleeding

19
Q

What is this? (INSERT PIC)

A

Severe Haemophilia and haemarthrosis

20
Q

What is this? (INSERT PIC)

A

Chest wall haemoarthroma

21
Q

What are the clinical complications of Haemophilia?

A
  • Synovitis
  • Chronic Haemophilic Arthropathy
  • Neurovascular compression (compartment syndromes)
  • Other sequelae of bleeding (Stroke)
22
Q

What is this? INSERT PIC

A

Severe haemophilia arthropathy

23
Q

How is Haemophilia diagnosed?

A
  • Clinical (Suspicions from history)
    *1st do routine coagulation screening
  • Isolated prolonged AP TT
  • Normal PT
  • Reduced F-Vlll or FIX (8 = Haemophilia A, 9 = Haemophilia B, intrinsic issues as these at clotting factors)

Genetic analysis (to confirm and offer to other family members)

24
Q
A

Haemophilia Treatment
Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement

25
Q
A

Haemophilia treatment (complications)
• Viral infection
• Inhibitors
D DAV P
HIV
HBV, HCV,
Others/ vCJD?
Anti FVIII Ab
Rare in FIX
Ml
Hyponatraemia(babies)

26
Q
A

von Willebrand Disease
Common (1 in 200)
Variable severity
Autosomal
Platelet Type bleeding (mucosal)
Quantitative and qualitative abnormalities of vWF

27
Q

Pic

A
28
Q
A

Von Willebrand Disease
• Type 1 quantitative deficiency
• Type 2 (A,B,M,N) qualitative deficiency determined by the site of mutation in relation
to vWF function
• Type 3 severe (complete) deficiency
DI 02 0-03
04 Cl CZ

29
Q
A

Von Willebrand Disease
• vWF concentrate or DDAVP
• Tranexamic Acid
• OCP etc

30
Q
A

Acquired Bleeding Disorders
Thrombocytopenia
Liver failure
Renal failure
• DIC
Drugs Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Apixaban, Dabigatran,
Bivalirudin
Acquired clotting factors deficiencies - rare

31
Q
A

Thrombocytopenia
• Decreased production
• Increased consumption
Marrow failure
Aplasia
Infiltration
Immune ITP
DIC
Hypersplenism

32
Q
A

Thrombocytopenia Clinical
Petechia
Ecchymosis
Mucosal Bleeding
Rare CNS bleeding

33
Q
A

Significant blood blisters - single platelets count

34
Q
A

Petechial rashes - urgent platelet count if seen

35
Q
A

• Adults vs Children
• Associations
Infection esp EBV,HIV
Lymphoma
Drug induced
• Blood picture = isolated thrombocytopenia
• Marrow ?
• Steroids, IV lgG, Thrombopoietin analogues (Eltrombopag and romiplostim),
Splenectomy - rare these days

36
Q
A

Liver Failure
• Factor l, Il, V, Vil, X, Xl
Prolonged P T, AP TT Reduced Fibrinogen
• Cholestasis - Vit K dept factor deficiency
Factor Il, Vil, X
Procoagulant
Factors
Anticoagulant
Factors
Normal Hemostasis
Liver
isease
Fiøvte The cf end

37
Q
A

Coagulation in liver disease

38
Q
A

Haemorrhagic Disease of the Newborn
Immature Coagulation Systems
Vitamin K deficient diet (esp Breast)
Fatal and incapacitating haemorrhage
Completely preventable by administration of vitamin K at birth (I.M vs P.O)