🥵 HARD

Blood is thicker than water or so the saying goes. Test yourself on this set of questions about the patient with bleeding tendency.

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Bleeding tendency

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Question 1

Stem 1, question 1 of 5

Bartholomew, a 6 year old male, presents to his GP, accompanied by his Father,  with fatigue and a swollen, painful knee, caused by him tripping over at school.

Haemophilia A is a deficiency of which clotting factor?
A
VII
B
VIII
C
IX
D
X
E
XI
Question 1 Explanation: 
Haemophilia A is a rare inherited condition results in a deficiency of factor VIII. Haemophilia B is a similar condition but is caused by a deficiency of factor IX.
Question 2

Stem 1, question 2 of 5

What is the inheritance pattern of Haemophilia A?
A
Autosomal Dominant
B
Autosomal Recessive
C
Mitochondrial
D
X-linked dominant
E
X-linked recessive
Question 2 Explanation: 
Haemophilia A is classically an X-linked recessive condition, however up to a third of those diagnosed with this disease have no family history and thus is caused by a de novo mutation. This is a good resource to understand more about haemophilia: https://www.hemophilia.org/Bleeding-Disorders/Types-of-Bleeding-Disorders/Hemophilia-A
Question 3

Stem 1, question 3 of 5

How does Haemophilia A lead to joint destruction?
A
Recurrent episodes of spontaneous bleeding into a joint causes invasive pannus formation that results in bone erosion
B
Recurrent episodes of HLA-B27 locus activation leads to an autoimmune mediated destruction of a joint
C
Inappropriate deposition of fibrin with the consumption fo coagulation factors and platelets
D
Degradation of cartilage leads to bone remodelling resulting in deformities
E
Recurrent episodes of spontaneous bleeding into a joint destroys the avascular cartilage on the articular surfaces
Question 3 Explanation: 
This question is outlining the pathophysiology of haemophilic arthropathy or recurrent haemarthroses. Cartilage found in joints is avascular and so is destroyed by the presence of blood in addition to hypertrophy of the synovium which erodes the cartilage and bone. This is a classical feature of Haemophilia and is often what causes someone to present initially.
Question 4

Stem 1, question 4 of 5

Which of the following would not be an investigation result seen in a person with Haemophilia A?
A
Abnormal Factor VIII clotting assay
B
Abnormal PFA-100 result
C
Normal platelet count
D
Normal prothrombin time
E
Prolonged activated partial thromboplastin time
Question 4 Explanation: 
PFA-100 is a method of assessing platelet function. In haemophilia platelet are functioning normally and thus you would not expect to see an abnormal PFA-100 result. All the other answers are expected investigation results for someone with haemophilia A.
Question 5

Stem 1, question 5 of 5

Which of the following is a suitable treatment for haemophilia A?
A
Apixaban
B
Anti-fibrinolytics
C
Heparin
D
Factor VIII replacement therapy
E
Factor IX replacement therapy
Question 5 Explanation: 
As Haemophilia is a deficiency of factor VIII a treatment is replace this missing factor. Anti-fibrinolytics can be used in disorders of primary haemostasis. Factor IX replacement is used in Haemophilia B (Christmas Disease). Apixaban and heparin are anti-coagulants
Question 6

Stem 2, question 1 of 5

Bettina, a 26 year old female, presents to her GP with menorrhagia and a recent prolonged bleeding episode following routine dental work

What is the most likely diagnosis?
A
Anti-thrombin deficiency
B
Disseminated intravascular coagulation
C
Factor V Ledien
D
Vitamin K deficiency
E
Von Willebrand Disease
Question 6 Explanation: 
Von Willebrand disease is the most common inherited bleeding disorder and often presents with heavy menstrual bleeding in people with a uterus and often prolonged bleeding following minor procedures such as dental work. Vitamin K deficiency can cause bleeding tendency but is much less common in this demographic.
Question 7

Stem 2, question 2 of 5

Bettina is diagnosed with Von Willebrand disease, which of the following is NOT an expected investigation result?
A
Defective platelet aggregation
B
Normal platelet count
C
Low Von Willebrand factor
D
Prolonged prothrombin time
E
Prolonged activated partial thromboplastin time
Question 7 Explanation: 
Von Willebrand disease is a deficiency of Von Willebrand factor which is needed for platelet aggregation in primary haemostasis, thus the platelet aggregation will be abnormal, but the number of platelet will be normal. Prolonged activated partial thromboplastin time is also often seen as Factor VIII levels are often low too (but not to the extent of haemophilia A)
Question 8

Stem 2, question 3 of 5

Years later Bettina has a vaignal delivery of her first child, however, has a post-partum haemorrhage. Which of the following drugs can be given to reduce the bleeding due to her Von Willebrand's disease?
A
Goserelin
B
Tranexamic acid
C
Nivolumab
D
Pamidronate
E
Ferrous sulphate
Question 8 Explanation: 
Goserlin is a luteinising hormone release hormone receptor antagonist. Tranexamic acid is anti-fibrinolytic that will prevent fibrin degradation and thus allow the platelet and haemostatic plug to remain for longer. Nivolumab is a PD-1 inhibitor used in melanoma. Pamidronate is a bisphosphonate. Ferrous sulphate is iron used in iron deficiency anaemia.
Question 9

Stem 2, question 4 of 5

Bettina's child develops haemorrhagic disease of the newborn, which pathology is this child at risk?
A
Biliary obstruction
B
Patent ductus arteriosus
C
Intracranial haemorrhage
D
Patent processes vaginalis
E
Intussception
Question 9 Explanation: 
Hopefully, this was a slightly more straightforward question! A haemorrhagic diseases leads to, you guessed it, a haemorrhage! This disease greatly increases the risk of an intracranial haemorrhage which can be catastrophic and may lead to death.
Question 10
What is the prophylaxis for  haemorrhagic disease of the newborn?
A
Vitamin K injection
B
Oxygen therapy
C
Blood transfusion
D
Fresh frozen plasma
E
Von Willebrand factor
Question 10 Explanation: 
Haemorrhagic disease of the newborn is due to a deficiency of vitamin K and as a direct result vitamin K dependent factors, thus there is a very simple treatment and prophylaxis, vitamin K! This is a national programme in which all neonates are offered a vitamin K injection to prevent this disease which has resulted in a reduction in the condition
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