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Immunology and Haematology Brian > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What should be asked when taking a history of bleeding?

A
  • Has the patient actually got a bleeding disorder
  • Severity of bleeding
  • Pattern of bleeding
  • Congenital or acquired
  • Mode of inheritance
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2
Q

What are questions to ask about the history of bleeding?

A
  • Bruising
  • Epistaxis (acute haemorrhage from nostril – bleeding nose)
  • Surgical procedure
    • If was bleeding after
  • Menorrhagia (abnormally heavy periods)
  • Post-partum haemorrhage
  • Post-trauma
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3
Q

What is epistaxis?

A

Acute haemorrhage from nostril - bleeding nose

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4
Q

What needs to be considered when thinking about the severity of bleeding?

A
  • How appropriate is bleeding for what happened
  • Ask what it takes to make them bleed
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5
Q

What are the 2 patterns of bleeding?

A
  • Platelet type
    • Mucosal
    • Epistaxis
    • Purpura
    • Menorrhagia
    • GI
  • Coagulation factor
    • Articular
    • Muscle haematoma
    • CNS
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6
Q

What are examples of platelet type bleeding?

A
  • Mucosal
  • Epistaxis
  • Purpura
  • Menorrhagia
  • GI
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7
Q

What are examples of coagulation factor bleeding?

A
  • Articular
  • Muscle haematoma
  • CNS
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8
Q

What should be done to skin lesions found on examination?

A
  • Check if blanch when pressed on
    • Petechia do not
    • Any lesion to do with blood vessel, such as spider naevi, do blanch
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9
Q

Do skin lesions related to blood vessels blanch or not blanch?

A

They blanch

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10
Q

How do you determine if bleeding is congenital or acquired?

A
  • Previous episodes?
  • Age at first event
  • Previous surgical challenges
  • Associated history

To investigation pattern of inheritance if congenital:

  • Family members with similar history
  • Sex
    • X linked only has affected males, female carrier
    • Autosomal affects females and males
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11
Q

What are some common bleeding conditions?

A
  • Haemophilia A and B
  • Von Willebrand disease
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12
Q

What are the 2 kinds of haemophilia?

A

Haemophilia A = factor XIII deficiency

Haemophilia B = factor IX deficiency

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13
Q

What is the inheritance of haemophilia?

A
  • X linked
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14
Q

Describe the epidemiology of haemophilia?

(incidence)

A
  • 1/10000 for A
  • 1/60000 for B
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15
Q

What form of haemophilia is more common?

A
  • 1/10000 for A
  • 1/60000 for B
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16
Q

What does severity of haemophilia depend on?

A

Severity of bleeding depends on the residual coagulation factor activity:

  • <1% is severe disease
    • Unprovoked bleeding into joints and muscles
  • 1-5% moderate disease
    • Will not bleed spontaneously but bleed excessively
  • 5-30% mild disease
    • Will not bleed spontaneously but bleed after things such as surgery and biopsy
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17
Q

Describe “severe”, “moderate” and “mild” haemophilia?

A
  • <1% is severe disease
    • Unprovoked bleeding into joints and muscles
  • 1-5% moderate disease
    • Will not bleed spontaneously but bleed excessively
  • 5-30% mild disease
    • Will not bleed spontaneously but bleed after things such as surgery and biopsy
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18
Q

What are the clinical features of haemophilia?

A
  • Haemarthrosis
  • Muscle haematoma
  • CNS bleeding
  • Retroperitoneal bleeding
  • Post-surgical bleeding
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19
Q

What is haemarthrosis?

A

Haemorrhage into a joint space, can be regarded as a subtype of joint effusion

20
Q

What are possible complications of haemophilia?

A
  • Synovitis
  • Chronic haemophilic arthropathy
  • Neurovascular compression (compartment syndromes)
  • Other sequelae of bleeding (stroke)
21
Q

Describe the investigations for haemophilia/how it is diagnosed?

A
  • Clinical
  • Routine coagulation tests
    • Prolonged APTT
      • Due to reduced FVIII or FIX
    • Normal prothrombin time (PT)
  • Assays to look at
    • Factor VIII and factor IX
  • Genetic analysis
22
Q

Describe the treatment for haemophilia?

A
  • Coagulation factor replacement
    • FVIII or FIX
    • Recombinant products – meaning factor is independent from donor
  • DDAVP
    • Analogue of vasopression
    • Indications – mild haemophilia A
  • Tranexamic acid
    • Effect – reduces rate of fibrinolysis
  • Prophylaxis in severe haemophilia
  • Gene therapy for future
  • Management of haemophilic arthropathy and muscles
    • Splints
    • Physiotherapy
    • Analgesia
    • Synovectomy
    • Joint replacement
23
Q

What is the effect of tranexamic acid?

A
  • Effect – reduces rate of fibrinolysis
24
Q

Describe the management of haemophilic arthropathy?

A
  • Splints
  • Physiotherapy
  • Analgesia
  • Synovectomy
  • Joint replacement
25
Q

What does arthropathy mean?

A

Disease of the joints

26
Q

What are possible complications for haemophilia treatment?

A
  • Viral infections
    • HIV, HBV, HCV
  • Development of inhibitory antibodies
    • Anti FVIII antibody
    • Rare in FIX
  • From DDAVP
    • MI
    • Hyponatraemia (babies)
27
Q

What is DDAVP?

A
  • Analogue of vasopression
  • Indications – mild haemophilia A
28
Q

Describe the epidemiology of von Willebrand disease?

(incidence)

A
  • Most common 1/200
29
Q

What is the most common bleeding disorder?

A

von Willebrand disease

30
Q

Describe the inheritance of von Willebrand disease?

A
  • Autosomal inheritance
31
Q

Describe the pathophysiology of von Willebrand disease?

A
  • Due to quantitative and qualitative abnormalities of vWF
32
Q

What are the different classes of von Willebrand disease?

A
  • Type 1
    • Quantitative deficiency
  • Type 2
    • Qualitative deficiency determined by the site of mutation in relation to vWF function
  • Type 3
    • Severe (complete) deficiency
33
Q

Describe the presentation of von WIllebrand disease?

A
  • Platelet type bleeding (mucosal)
34
Q

What pattern of bleeding is present in von Willebrand disease?

A
  • Platelet type bleeding (mucosal)
35
Q

Describe the treatment for von Willebrand disease?

A
  • vWF concentrate or DDAVP
    • Contraindications for DDAVP – cardiac conditions, young children
  • Tranexamic acid
  • Topical applications
    • Dressings in dental situations
  • Combined oral contraceptive pill
    • Indication – menorrhagia
36
Q

What are examples of acquired bleeding disorders?

A
  • Thrombocytopenia
  • Liver failure
  • Renal failure
  • DIC
  • Drugs
    • Warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin…
37
Q

What are some drugs that can cause acquired bleeding disorders?

A
  • Warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin…
38
Q

Describe the aetiology of thrombocytopenia?

A
  • Decreased production
    • Bone marrow failure
    • Aplasia
    • Infiltration
  • Increased consumption
    • Immune ITP (idiopathic thrombocytopenic purpura)
    • Non-immune DIC
    • Hypersplenism
39
Q

Describe the presentation of thrombocytopenia?

A
  • Petechia
  • Ecchymosis
  • Mucosal bleeding
  • Rare CNS bleeding
40
Q

What is idiopathic thrombocytopenic purpura associated with?

A
  • Infection
    • Especially EBV, HIV
  • Collagenosis
    • Connective tissue diseases
  • Lymphoma
  • Drug induced
41
Q

Describe the treatment for idiopathic thrombocytopenic purpura?

A
  • Steroids
    • Elevate platelet count
  • IgG IV
  • Thrombopoietin analogues (eltromobag, rombipostim)
42
Q

Describe the pathophysiology of liver failure in relation to bleeding?

A
  • Liver produces
    • Factors I, II, V, VII, VIII, IX, X, XI – procoagulants
    • But also produces anticoagulants
43
Q

Describe the presentation of liver failure in relation to bleeding?

A
  • Prolonged PT
  • APTT reduced fibrinogen
  • Cholestasis
44
Q

Describe the treatment for liver failure in relation to bleeding?

A
  • Replacement FFP
  • Vitamin K
45
Q

Describe the pathophysiology of haemorrhagic disease of new-born?

A
  • Newborns have immature coagulation systems, particularly low in vitamin K dependent factors
    • II, VII, IX and X
    • Protein C and protein S
46
Q

Describe the management of haemorrhagic disease of new-born?

A
  • Prophylaxis
    • Completely preventable by administration of vitamin K at birth

Immunology and Haematology Brian (23 decks)

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