Bleeding Disorders Flashcards
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
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
What is epistaxis?
Acute haemorrhage from nostril - bleeding nose
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
What are the 2 patterns of bleeding?
- Platelet type
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI
- Coagulation factor
- Articular
- Muscle haematoma
- CNS
What are examples of platelet type bleeding?
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI
What are examples of coagulation factor bleeding?
- Articular
- Muscle haematoma
- CNS
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
Do skin lesions related to blood vessels blanch or not blanch?
They blanch
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
What are some common bleeding conditions?
- Haemophilia A and B
- Von Willebrand disease
What are the 2 kinds of haemophilia?
Haemophilia A = factor XIII deficiency
Haemophilia B = factor IX deficiency
What is the inheritance of haemophilia?
- X linked
Describe the epidemiology of haemophilia?
(incidence)
- 1/10000 for A
- 1/60000 for B
What form of haemophilia is more common?
- 1/10000 for A
- 1/60000 for B
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
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
What are the clinical features of haemophilia?
- Haemarthrosis
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post-surgical bleeding
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)
- Prolonged APTT
- 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
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
