Bleeding Disorders Flashcards by Brian Mendonsa

What should be asked when taking a history of bleeding?

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

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What are questions to ask about the history of bleeding?

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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What is epistaxis?

Acute haemorrhage from nostril - bleeding nose

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

How appropriate is bleeding for what happened
Ask what it takes to make them bleed

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What are the 2 patterns of bleeding?

Platelet type
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI
Coagulation factor
- Articular
- Muscle haematoma
- CNS

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What are examples of platelet type bleeding?

Mucosal
Epistaxis
Purpura
Menorrhagia
GI

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What are examples of coagulation factor bleeding?

Articular
Muscle haematoma
CNS

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What should be done to skin lesions found on examination?

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

They blanch

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How do you determine if bleeding is congenital or acquired?

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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What are some common bleeding conditions?

Haemophilia A and B
Von Willebrand disease

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What are the 2 kinds of haemophilia?

Haemophilia A = factor XIII deficiency

Haemophilia B = factor IX deficiency

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What is the inheritance of haemophilia?

X linked

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Describe the epidemiology of haemophilia?

(incidence)

1/10000 for A
1/60000 for B

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What form of haemophilia is more common?

1/10000 for A
1/60000 for B

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What does severity of haemophilia depend on?

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

<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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What are the clinical features of haemophilia?

Haemarthrosis
Muscle haematoma
CNS bleeding
Retroperitoneal bleeding
Post-surgical bleeding

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What is haemarthrosis?

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

What are possible complications of haemophilia?

Synovitis
Chronic haemophilic arthropathy
Neurovascular compression (compartment syndromes)
Other sequelae of bleeding (stroke)

Describe the investigations for haemophilia/how it is diagnosed?

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

Describe the treatment for haemophilia?

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

What is the effect of tranexamic acid?

Effect – reduces rate of fibrinolysis

Describe the management of haemophilic arthropathy?

Splints
Physiotherapy
Analgesia
Synovectomy
Joint replacement

What does arthropathy mean?

Disease of the joints

What are possible complications for haemophilia treatment?

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

What is DDAVP?

* Analogue of vasopression * Indications – mild haemophilia A

Describe the epidemiology of von Willebrand disease? (incidence)

* Most common 1/200

What is the most common bleeding disorder?

von Willebrand disease

Describe the inheritance of von Willebrand disease?

* Autosomal inheritance

Describe the pathophysiology of von Willebrand disease?

* Due to quantitative and qualitative abnormalities of vWF

What are the different classes of von Willebrand disease?

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

Describe the presentation of von WIllebrand disease?

* Platelet type bleeding (mucosal)

What pattern of bleeding is present in von Willebrand disease?

* Platelet type bleeding (mucosal)

Describe the treatment for von Willebrand disease?

* 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

What are examples of acquired bleeding disorders?

* Thrombocytopenia * Liver failure * Renal failure * DIC * Drugs * Warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin…

What are some drugs that can cause acquired bleeding disorders?

* Warfarin, heparin, aspirin, clopidogrel, rivaroxaban, apixaban, dabigatran, bivalirudin…

Describe the aetiology of thrombocytopenia?

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

Describe the presentation of thrombocytopenia?

* Petechia * Ecchymosis * Mucosal bleeding * Rare CNS bleeding

What is idiopathic thrombocytopenic purpura associated with?

* Infection * Especially EBV, HIV * Collagenosis * Connective tissue diseases * Lymphoma * Drug induced

Describe the treatment for idiopathic thrombocytopenic purpura?

* Steroids * Elevate platelet count * IgG IV * Thrombopoietin analogues (eltromobag, rombipostim)

Describe the pathophysiology of liver failure in relation to bleeding?

* Liver produces * Factors I, II, V, VII, VIII, IX, X, XI – procoagulants * But also produces anticoagulants

Describe the presentation of liver failure in relation to bleeding?

* Prolonged PT * APTT reduced fibrinogen * Cholestasis

Describe the treatment for liver failure in relation to bleeding?

* Replacement FFP * Vitamin K

Describe the pathophysiology of haemorrhagic disease of new-born?

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

Describe the management of haemorrhagic disease of new-born?

* Prophylaxis * Completely preventable by administration of vitamin K at birth