Bleeding Disorders Flashcards
Includes haemophilia and Von Willebrand disease
Haemophilia A is a deficiency of which clotting factor?
Factor VIII
Haemophilia B is a deficiency of which clotting factor?
Factor IX
Haemophilia results in excess bleeding because it makes primary haemostasis less effective. T/F?
False - Haemophilia results in excess bleeding because it makes secondary haemostasis less effective
What is the mode of inheritance for haemophilia A and B?
X-linked recessive
Which sex is primarily affected by haemophilia?
Males
It is an x-linked recessive condition
A family history of haemophilia is always present in patients with. haemophilia. T/F?
False - up to 30% of new cases are. the result of sporadic mutations
The likelihood of bleeding can be roughly predicted from the level of Factor VIII present in haemophilia A. How is severe haemophilia A classified?
Factor VIII level <2 units /dL
Frequent spontaneous bleeds into joints and muscles
The likelihood of bleeding can be roughly predicted from the level of Factor VIII present in haemophilia A. How is moderate haemophilia A classified?
Factor VIII level 2-5 units /dL
Some spontaneous bleeds. Bleeding after minor trauma
The likelihood of bleeding can be roughly predicted from the level of Factor VIII present in haemophilia A. How is mild haemophilia A classified?
Factor VIII level 5-45 units /dL
Excessive bleeding after significant trauma or surgery
At what stage in development does haemophilia A become apparent?
When the child begins to crawl
Haemarthrosis is the most common problem in haemophilia A. Which joints are most commonly affected by this?
Elbows and knees
What are the clinical features of haemophilia A?
Haemarthrosis Easy bruising (ecchymosis) Haematomas Prolonged bleeding after cuts GI bleeding Haematuria Severe nosebleeds (epitaxes)
What result would you expect to see on PT and APTT for a patient with haemophilia?
PT - normal
APTT - prolonged
Besides APTT, what test can be used to confirm haemophilia?
Clotting factor assay
Describe the management of haemophilia A?
Injections of Factor VIII concentrate which can often be self-administered during bleeds
Desmopressin is useful in mild haemophilia A