Bleeding Disorders Flashcards
What is haemorrhagic diathesis?
An unusual susceptibility to bleeding
What would you want to know in a history of bleeding?
- Bruising
- Epistaxis
- Post surgical bleeding
- Menorrhagia
- Post-partum haemorrhage
- Post-trauma
What patterns of bleeding would make a disorder of the platelets more common?
- Mucosal
- Epistaxis
- Purpura
- Menorrhagia
- GI bleeds
What patterns of bleeding would make a coagulation factor disorder more common?
- Articular
- Muscle haematoma
- CNS
What factors make a congenital disorder more likely?
- Previous episodes
- The age at first event
- Previous surgical challenges
- Associated history
Describe the features of haemophilia A and B
- X linked
- Identical phenotypes
- Severity of bleeding depends on the residual coagulation factor activity
- Severe <1%
- Moderate 1-5%
- Mild 5-30%
What are the clinical features of haemophilia?
- Haemarthrosis (hinge joints do much worse than ball and socket joints)
- Muscle haematoma
- CNS bleeding
- Retroperitoneal bleeding
- Post surgical bleeding
What are the complications of haemophilia?
- Synovitis
- Chronic haemophilic arthropathy
- Neurovascular compression
- Sequelae of bleeding (stroke etc.)
How can haemophilia be diagnosed?
- Clinical
- Prolonged APTT
- Normal PT
- Reduced FVIII or FIX
- Genetic analysis
How can the bleeding diathesis of haemophilia be treated?
- Coagulation factor replacement FVIII/IX
- DDAVP (desmopressin)
- Tranexamic acid
- Prophylaxis in severe haemophilia
- ?Gene therapy
How can haemophilia be managed?
- Splints
- Physio
- Analgesia
- Synovectomy
- Joint replacement
What complications can haemophilia treatment have?
- Viral infection
- Inhibitors: anti FVIII antibodies etc.
- DDAVP can cause MI and hyponatraemia
Describe the features of von Willebrand disease
- Variable severity
- Autosomal
- Platelet type bleeding
List the three different types of Von Willebrand Disease
- Type 1: quantitative deficiency
- Type 2: qualitative deficiency determined by the site of the mutation
- Type 3: severe (complete) deficiency
How can Von Willebrand disease be treated?
- vWF concentrate or DDAVP
- Tranexamic acid
- Topical applications
- OCP etc.
Give examples of acquired bleeding disorders
- Thrombocytopenia
- Liver failure
- Renal failure
- DIC
- Those caused by drugs
What are the causes of thrombocytopenia?
- Decreased production: marrow failure, aplasia and infiltration
- Increased consumption: immune ITP, non immune DIC and hypersplenism
What is the clinical presentation of thrombocytopenia?
- Petechia
- Ecchymosis
- Mucosal bleeding
- Rare CNS bleeding
What are the causes of immune thrombocytopenia?
- Infections
- Collagenosis
- Lymphoma
- Drug induced
- Blood isolated
How can ITP be treated?
- Steroids
- IgG
- Splenectomy
- Thrombopoietin analogues (eltrombopag and romiplostim)
What effect does liver failure have on clotting and how can it be treated?
- It causes a reduction in the factors
- Prolonged PT, APTT and reduced fibrinogen
- Cholestasis and vit K factor deficiency
- Managed by replacement FFP and vitamin K
What is haemorrhagic disease of the newborn and how can it be treated?
- Immature coagulation systems
- Vitamin K deficient diet
- Fatal and incapacitating haemorrhage
- Preventable by administration of vitamin K at birth
