Bleeding disorders Flashcards
What is haemorrhagic diathesis?
Any quantitative or qualitative abnormality in blood resulting in inhibition of function of platelets, vWF or coagulation factors.
What are some key things you need to establish about the history of bleeding?
Bruising Epistaxis Post- surgical Menorrhagia Post-partum haemorrhage Post-trauma
What are the different patterns of bleeding for platelet inhibition?
Muscosal Epistaxis (nosebleed) Purpura Menorrhagia GI
What are the different patterns if bleeding for coagulation factor inhibition?
Articular
Muscle haematoma
CNS
What are the genetic features of haemophilia A and B?
x-linked Identical phenotypes 1 in 10,000 and 1 in 60,000 Severity of bleeding depends on 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 haemophilia diagnosed?
Clinically. Prolonged APTT Normal PT Reduced Factor 8 or 9 Genetic analysis
What is the treatment for haemophilia bleeding diathesis?
Coagulation factor replacement - factor 8/9
Recombinant products
DDAVP
Tranexamic acid
Emphasis on prophylaxis in severe haemophilia.
Gene therapy?
What is the treatment foe haemophilia?
Splints Physiotherapy Analgesia Synovectomy Joint replacement
What are the complications of haemophilia treatment?
Viral infection - HIV, HB, HCV etc.
Inhibitors can cause anti-factor8 antibodies.
DDAVP - MI, hyponatraemia in babies
What are some features of Von Willebrand disease?
Common 1 in 200 Variable severity Autosomal Platelet the bleeding (mucosal) Quantitative and qualitative abnormalities of vWF
What are the different types of vWD?
Type 1 quantitative deficiency Type 2 (A,B,M,N)qualitative deficiency determined by site of mutation in relation to vWF function. Type 3 severe (complete deficiency).
What is the treatment for vWD?
vWF concentrate or DDAVP
Tranexamic acid
Topical applications
OCP etc
What are some examples of acquired bleeding disorders?
Thrombocytopenia Liver failure Renal failure DIC Drugs - warfarin, heparin, aspirin, clopidogrel etc.
What are the characteristics of Thrombocytopenia?
Decreased production resulting in marrow failure, aplasia, infiltration.
Increased consumption - immune ITP, non immune DIC, hypersplenism.
What are some clinical signs of thrombocytopenia?
Petechia
Ecchymosis - discolouration of skin due to bleeding underneath
Mucosal bleeding
Rare CNS bleeding
What is ITP?
Immune thrombocytopenic purpura (ITP) is a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting.
What is ITP associated with?
Infection especially EBV, HIV
Collagenosis
Lymphoma
Drug induced
What are some treatment options for ITP?
Steroids
IV IgG
Splenectomy
Thrombopoietin analogues e.g. eltrombopag, romiplostim.
What are some causes of liver failure?
Factor 1,2,5,7,8,9,10 and 11.
Prolonged PT, APTT
Reduced fibrinogen.
What are some features of HAemorrhagic disease of the Newborn?
Immature coagulation systems normally due to a vitamin K deficient diet.
Can result in fatal and incapacitating haemorrhage.
Completely preventable by administration of vitamin K at birth.
