Bleeding Disorder Flashcards
How is platelet type bleeding characterised?
Mucosal (petechiae + bruising, platelet count <10) Epistaxis Purpura Menorrhagia GI bleeds (rare)
How is coagulation factor bleeding characterised?
Articular haemorrhage (presents with hot, swollen, fluid filled joint- commonly knee or ankle) Muscle Haematoma CNS (intracranial haemorrhage)
What type of inheritance is associated with haemophilia?
X-linked
How is severity of haemophilia characterised?
Severity of bleeding depends on the residual coagulation factor activity as follows:
•Severe <1% residual activity (will bleed with no provocation)
•Moderate 1-5% residual activity (will profusely but only with provocation)
•Mild 5-30% residual activity
What are the clinical features of haemophilia?
Haemarthrosis (usually weight bearing and hinge joints so most commonly ankle then knee)
Muscle haematoma
CNS bleeding (very rare)
Retroperitoneal bleeding (very rare)
Post surgical bleeding (can be planned and prevented, 7-10 days of treatment before major operations)
What are the clinical complications of haemophilia?
Synovitis
Chronic Haemophilic Arthropathy
Neurovascular compression (compartment syndromes)
Other consequences of bleeding (Stroke)
How is haemophilia diagnosed?
Clinical Prolonged APTT Normal PT Reduced FVIII or FIX Genetic analysis 1/3 of X-linked conditions are new mutations so family history might not be present
How is haemophilia treated?
With regards to bleeding diathesis: Coagulation factor replacement FVIII/IX (now almost entirely recombinant products) DDAVP (desmopressin- results in release of store vWF Tranexamic Acid Wider treatment of haemophilia: Splints Physiotherapy Analgesia Synovectomy Joint replacement
What are the possible complications of haemophilia treatment?
Viral infection- HIV, HBV, HCV
Inhibitors- anti FVIII Ab (only in severe cases where there is zero antigen, usually occurs as babies and immune system will become tolerant with gradual introduction over time), rare in FIX
Desmopressin- contraindicated by cardiac history due to MI risk, can’t be used in babies as it will cause hyponatremia
What are the three types of von Willebrand’s disease?
Type 1 quantitative deficiency
Type 2 qualitative deficency determined by the site of mutation in relation to vWF function
Type 3 severe (complete) deficiency
What is the pathological cause of von Willebrand’s disease?
Quantitative and qualitative abnormalities of von Willebrand’s factor
How is von Willebrand’s disease treated?
vWF concentrate or DDAVP
Tranexamic Acid
Topical applications
OCP
What are the causes of acquired bleeding disorders?
Thrombocytopenia Liver failure Renal failure DIC Drugs
What are the possible causes of thrombocytopenia?
Decreased production (marrow failure, aplasia, infiltration) or increased consumption (immune ITP, non immune DIC, hypersplenism) of platelets
What are the clinical features of thrombocytopenia?
Petechia
Ecchymosis
Mucosal Bleeding
Rare CNS bleeding
What can idiopathic thrombocytopenic purpura be associated with?
Infection Collagenosis Lymphoma Drug induced Blood isolated thrombocytopenia, possibly due to marrow failure
How is idiopathic thrombocytopenic purpura treated?
Steroids
IV IgG
Splenectomy
Thrombopoietin analogues
What are the characteristics of haemorrhagic disease of the newborn?
Immature Coagulation Systems
Vitamin K deficient diet (esp Breast, vit K injection given to baby at birth and eliminates risk of HDN)
Fatal and incapacitating haemorrhage
Completely preventable by administration of vitamin K at birth (I.M vs P.O)
What are the haematological complications of liver failure?
Associated with higher risks of both bleeding and thrombosis. Prolonged PT, APTT and reduced fibrinogen is often associated with liver failure
What can be given to reduce blood related risks in liver failure?
Replacement FFP
Vitamin K
What is the deficiency associated with haemophilia A?
Factor VIII deficiency
What is the deficiency associated with haemophilia B?
Factor IX deficiency
