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Systems: Haematology + Immunology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What are the components of normal haemostasis?

A
  • Vessel wall
  • Platelets
  • von Willebrand factor
  • Coagulation factors
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2
Q

What is the normal primary haemostatic response?

A

Platelet plug formation

  • Platelets
  • vWF
  • Wall
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3
Q

What is the normal secondary haemostatic response?

A

Fibrin plug formation

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4
Q

What causes haemorrhagic diathesis?

A

Any quantitative or qualitative abnormality or inhibition of function of:

  • Platelets
  • vWF
  • Coagulation factors
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5
Q

What should be established from 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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6
Q

What pay somebody with a history of bleeding present with?

A
  • Bruising
  • Epistaxis
  • Post-surgical bleeding
  • Menorrhagia
  • Post-partum haemorrhage
  • Post-trauma
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7
Q

How do you establish the severity of bleeding?

A

How appropriate is the bleeding?

  • Amount lost compared to injury
  • Bleeding with no provocation
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8
Q

What is platelet type bleeding?

A
  • Mucosal
  • Epistaxis
  • Purpura
  • Menorrhagia
  • GI
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9
Q

What is coagulation factor type bleeding.?

A
  • Articular
  • Muscle haematoma
  • CNS
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10
Q

How do you determine whether a disorder is congenital or acquired?

A
  • Previous episodes of bleeding (lack of post surgical bleeding on prior procedures suggests acquired)
  • Age at first event
  • Previous surgical challenged
  • Associated history
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11
Q

What factors would suggest hereditary disorder?

A
  • Family members with similar history

- Sex

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12
Q

What type of inheritance does haemophilia have?

A

X-linked

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13
Q

What is the difference in phenotype between haemophilia A and B?

A

No difference, they have identical phenotypes

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14
Q

What is the prevalence of haemophilia?

A
  • A 1 in 10,000

- B 1 in 60,000

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15
Q

What does the severity of bleeding in haemophilia depend on?

A

Severity of bleeding depends on the residual coagulation factor activity

  • <1% severe
  • 1-5% moderate
  • 5-30% mild
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16
Q

What are the clinical features of haemophilia?

A
  • Haemarthrosis
  • Muscle haematoma
  • CNS bleeding
  • Retroperitoneal bleeding
  • Post surgical bleeding
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17
Q

What are the clinical complications of haemophilia?

A
  • Synovitis
  • Chronic Haemophilic Arthropathy
  • Neurovascular compression (compartment syndromes)
  • Other sequelae of bleeding (Stroke)
18
Q

How is a diagnosis of haemophilia made?

A
  • Clinical
  • Prolonged APTT
  • Normal PT
  • Reduced FVIII or FIX
  • Genetic analysis
19
Q

How is bleeding diathesis of haemophilia treated?

A
  • Coagulation factor replacement FVIII/IX
  • Now almost entirely recombinant products
  • DDAVP
  • Tranexamic Acid
  • Emphasis on prophylaxis in severe haemophilia
  • Gene therapy?
20
Q

How is haemophilia treated?

A
  • Splints
  • Physiotherapy
  • Analgesia
  • Synovectomy
  • Joint replacment
21
Q

What are the complications associated with haemophilia treatment?

A

Viral infection

  • HIV
  • HBV, HCV
  • Others/ vCJD

Inhibitors

  • Anti FVIII Ab
  • Rare in FIX

DDAVP

  • MI
  • Hyponatraemia
22
Q

What is the prevalence of von Willebrand disease?

A

Common, 1 in 200

23
Q

What is the inheritance of von Willebrand disease?

A

Autosomal

24
Q

How does von Willebrand disease present?

A
  • Platelet type bleeding(mucosal)

- Variable severity

25
Q

What is von Willebrand disease?

A

Quantitative and qualitative abnormalities of vWF

26
Q

What are the different types of von Willebrand disease?

A
  • Type 1 quantitative deficiency
  • Type 2 (A,B,M,N) qualitative deficency determined by the site of mutation in relation to vWF function
  • Type 3 severe (complete) deficiency
27
Q

What is the treatment for von Willebrand disease?

A
  • vWF concentrate or DDAVP
  • Tranexamic Acid
  • Topical applications
  • OCP etc
28
Q

Give examples of acquired bleeding disorders.

A
  • Thrombocytopenia
  • Liver failure
  • Renal failure
  • DIC
  • Drugs Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Apixaban, Dabigatran, Bivalirudin
29
Q

Thrombocytopenia

A

Abnormally low levels of platelets

30
Q

What can cause thrombocytopenia?

A

Decreased production

  • Marrow failure
  • Aplasia
  • Infiltration

Increased consumption

  • Immune ITP
  • Non immune DIC
  • Hypersplenism
31
Q

How does thrombocytopenia present clinically?

A
  • Petechia
  • Ecchymosis
  • Mucosal Bleeding
  • Rare CNS bleeding
32
Q

What is ITP associated with?

A
  • Infection esp, EBC, HIV
  • Collagenosis
  • Lymphoma
  • Drug induced
33
Q

How is ITP diagnosed?

A
  • Blood isolated thrombocytopenia

- Marrow (rare)

34
Q

How is ITP managed?

A
  • Steroids
  • IV IgG
  • Splenectomy
  • Thrombopoietin analogues (eltrombopag and romiplostim)
35
Q

How are blood disorders caused by liver failure treated?

A
  • Replacement FFP

- Vitamin K

36
Q

What factors are deficient in liver failure?

A

II, VII, IX, X

37
Q

What results would be seen in liver failure?

A

-Prolonged PT, APTT and reduced fibrinogen

38
Q

What does most bleeding in liver failure occur as a result of?

A

Structural lesions including varices

39
Q

What are the features of haemorrhagic disease of the new-born?

A
  • Immature Coagulation Systems
  • Vitamin K deficient diet (esp Breast)
  • Fatal and incapacitating haemorrhage
40
Q

How is haemorrhagic disease of the new born prevented?

A

Completely preventable by administration of vitamin K at birth (I.M vs P.O)

Systems: Haematology + Immunology (19 decks)

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