Bleeding Disorders Flashcards
What are the components of normal haemostasis?
- Vessel wall
- Platelets
- von Willebrand factor
- Coagulation factors
What is the normal primary haemostatic response?
Platelet plug formation
- Platelets
- vWF
- Wall
What is the normal secondary haemostatic response?
Fibrin plug formation
What causes haemorrhagic diathesis?
Any quantitative or qualitative abnormality or inhibition of function of:
- Platelets
- vWF
- Coagulation factors
What should be established from a bleeding history?
- Has the patient actually got a bleeding disorder?
- How severe is the disorder?
- Pattern of Bleeding
- Congenital or Acquired
- Mode of inheritance
What pay somebody with a history of bleeding present with?
- Bruising
- Epistaxis
- Post-surgical bleeding
- Menorrhagia
- Post-partum haemorrhage
- Post-trauma
How do you establish the severity of bleeding?
How appropriate is the bleeding?
- Amount lost compared to injury
- Bleeding with no provocation
What is platelet type bleeding?
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI
What is coagulation factor type bleeding.?
- Articular
- Muscle haematoma
- CNS
How do you determine whether a disorder is congenital or acquired?
- Previous episodes of bleeding (lack of post surgical bleeding on prior procedures suggests acquired)
- Age at first event
- Previous surgical challenged
- Associated history
What factors would suggest hereditary disorder?
- Family members with similar history
- Sex
What type of inheritance does haemophilia have?
X-linked
What is the difference in phenotype between haemophilia A and B?
No difference, they have identical phenotypes
What is the prevalence of haemophilia?
- A 1 in 10,000
- B 1 in 60,000
What does the severity of bleeding in haemophilia depend on?
Severity of bleeding depends on the residual coagulation factor activity
- <1% severe
- 1-5% moderate
- 5-30% mild
What are the clinical features of haemophilia?
- Haemarthrosis
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post surgical bleeding
What are the clinical complications of haemophilia?
- Synovitis
- Chronic Haemophilic Arthropathy
- Neurovascular compression (compartment syndromes)
- Other sequelae of bleeding (Stroke)
How is a diagnosis of haemophilia made?
- Clinical
- Prolonged APTT
- Normal PT
- Reduced FVIII or FIX
- Genetic analysis
How is bleeding diathesis of haemophilia treated?
- Coagulation factor replacement FVIII/IX
- Now almost entirely recombinant products
- DDAVP
- Tranexamic Acid
- Emphasis on prophylaxis in severe haemophilia
- Gene therapy?
How is haemophilia treated?
- Splints
- Physiotherapy
- Analgesia
- Synovectomy
- Joint replacment
What are the complications associated with haemophilia treatment?
Viral infection
- HIV
- HBV, HCV
- Others/ vCJD
Inhibitors
- Anti FVIII Ab
- Rare in FIX
DDAVP
- MI
- Hyponatraemia
What is the prevalence of von Willebrand disease?
Common, 1 in 200
What is the inheritance of von Willebrand disease?
Autosomal
How does von Willebrand disease present?
- Platelet type bleeding(mucosal)
- Variable severity
What is von Willebrand disease?
Quantitative and qualitative abnormalities of vWF
What are the different types of von Willebrand disease?
- 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
What is the treatment for von Willebrand disease?
- vWF concentrate or DDAVP
- Tranexamic Acid
- Topical applications
- OCP etc
Give examples of acquired bleeding disorders.
- Thrombocytopenia
- Liver failure
- Renal failure
- DIC
- Drugs Warfarin, Heparin, Aspirin, Clopidogrel, Rivaroxaban, Apixaban, Dabigatran, Bivalirudin
Thrombocytopenia
Abnormally low levels of platelets
What can cause thrombocytopenia?
Decreased production
- Marrow failure
- Aplasia
- Infiltration
Increased consumption
- Immune ITP
- Non immune DIC
- Hypersplenism
How does thrombocytopenia present clinically?
- Petechia
- Ecchymosis
- Mucosal Bleeding
- Rare CNS bleeding
What is ITP associated with?
- Infection esp, EBC, HIV
- Collagenosis
- Lymphoma
- Drug induced
How is ITP diagnosed?
- Blood isolated thrombocytopenia
- Marrow (rare)
How is ITP managed?
- Steroids
- IV IgG
- Splenectomy
- Thrombopoietin analogues (eltrombopag and romiplostim)
How are blood disorders caused by liver failure treated?
- Replacement FFP
- Vitamin K
What factors are deficient in liver failure?
II, VII, IX, X
What results would be seen in liver failure?
-Prolonged PT, APTT and reduced fibrinogen
What does most bleeding in liver failure occur as a result of?
Structural lesions including varices
What are the features of haemorrhagic disease of the new-born?
- Immature Coagulation Systems
- Vitamin K deficient diet (esp Breast)
- Fatal and incapacitating haemorrhage
How is haemorrhagic disease of the new born prevented?
Completely preventable by administration of vitamin K at birth (I.M vs P.O)
