Flashcards in Heritable Bleeding Disorders Deck (28):
The balance between bleeding and clotting
What 2 mechanisms for the haemostatic plug?
Aggregation and coagulation
3 main classes of bleeding disorders
Which classes of bleeding disorders results in bleeding in the skin or mucosa
Which classes of bleeding disorders result in deep muscular joint bleeds following trauma?
What drug affects the normal function of platelets
What 2 diseases affect the vessel wall
What disease is the abnormal interaction between platelets and the vessel wall
Von Willebrand Disease
4 characteristics of bleeding disorders due to vascular/platelet defects
Petechiae and superficial bruises
Prolonged and non recurrents
4 characteristics of bleeding disorders due to a defect in coagulation
Deep spreading haematoma
Prolonged, often recurrent
Red spots on the skin that do not blanch with pressure and are not palpable
Is von willebrand disease AR or AD
What is the function of von willebrand factor?
Helps platelets adhere to vessel wall. Acts as a carrier for factor 8
Most common heritable bleeding disorder
Von Willebrand disease
Symptoms of von willebrand disease
Mucocutaneous bleeding including menorrhagia (heavy periods)
Post operative/post partem bleeding
If you are blood group O are you more or less likely to have von willebrand disease?
Treatment for von willebrand disease
Factor concentrations (including VWF)
DDAVP-desmopressin-releases VWF from stores inside endothelial cells
What is the most common of coagulation factor deficiencies?
Activated partial thromboplastin time measures which pathway and up until what factor?
Prothrombin time measures which pathway and up until what factor?
The thrombin clotting time measures what part of the clotting cascade
The euglobulin lysis time measures what part of the clotting cascade
Plasmin>fibrin degredation products
What inheritence pattern are the haemophilias?
X linked recessive
Haemophilia A is a deficiency in which factor
Haemophilia B is a deficiency in which factor
Which is more common, haemophilia A or B?
Is severity variable or constant between family members with haemophilia?