Clinical Cases - Haemostasis and Thrombosis Flashcards
- A 20 month old boy attends accident and emergency with his father
- The child has refused to walk for the past 2 days having been walking unsupported for 4 months
- The right knee is slightly swollen and feels warm to touch
- Blood is aspirated from the knee
Which of the following is the most likely diagnosis?
A. Acute lymphoblastic leukaemia
B. Osteomyelitis
C. Von Willebrand Disease
D. Haemophilia A
Diagnosis is most likely haemophilia
How do you differentiate between haemophilia A and haemophilia B?
A. Measure the APTT
B. Measure the PT
C. Measure factor VIII and IX levels
D. Do a platelet count
Measure the factor VIII and IX levels
Patients factor VIII level was 0%
Patients factor IX level was 73%
What is the most likely diagnosis?
Severe haemophilia A
How is haemophilia A inherited?
A. Autosomal Recessive
B. X-linked
C. Y-linked
D. Autosomal Dominant
X-linked
The mother tells you that her father also has haemophilia A and wants to know the chances of haemophilia in any further children born to her.
What is the chance of any further child having haemophilia A?
A. 1 in 2
B. Can’t say because it depends on the mothers status
C. 1 in 4
D. Random chance - not quantifiable
The chance is 1 in 4 as the mother is an obligate carrier of haemophilia
Having established the diagnosis of severe haemophilia A with a joint bleed, what optimal treatment should you give?
A. Fresh frozen plasma
B. Plasma derived Factor VIII
C. DDAVP and tranexamic acid
D. Recombinant factor VIII
Recombinant factor VIII
The father is concerned about the boy receiving factor VIII because of infections in his father-in-law related to factor VIII treatment
Which of these is not a complication of plasma derived factor VIII treatment?
A. Hepatitis B
B. HIV
C. Hepatitis C
D. Hepatitis A
Hepatitis A is not transmitted by concentrate
What is the other main complication of treatment of severe haemophilia A with factor VIII concentrates?
A. Allergic reactions
B. Increased risk of thrombosis
C. Development of inhibitory antibodies
D. Addiction to factor VIII
Biggest complication is development of inhibitory antibodies (About 20% of cases)
The boy should be treated with recombinant factor VIII
What is the best treatment regimen for these patients?
A. Treat the patient as soon as possible only after he has a bleed
B. Give factor VIII regularly once weekly
C. Give factor VIII at least 3 times weekly
D. Give factor VIII every day
E. Consider bone marrow transplantation
C. Give factor VIII at least three times weekly
or every 48 hours
Treatment regiment for patients needing recombinant factor IX
Twice weekly
- A 24 year old female student presents with a 4 day history of easy bruising and small red spots on her ankles
- She has no previous history of abnormal bleeding
- Previous tonsillectomy with no bleeding
- Recently been off colour with flu like symptoms
- Only medication COCP
What are these non-blanching spots on her arms?
A. Spider naevi
B. Bruises
C. Petechiae
D. Telangectasia
Petechiae
Case 2
Which of these symptoms is she unlikely to have?
A. Menorrhagia
B. Swollen, hot painful leg
C. Epistaxis
D. Buccal bleeding
She has petechiae, menorrhagia, epistaxis easy bruising, and blood blisters in her mouth
She is least likely to have a swollen leg
Case 2
She has petechiae, menorrhagia, epistaxis easy bruising, and blood blisters in her mouth.
What is the likely cause of her symptoms?
A. Thrombocytopenia
B. Scurvy
C .Vitamin K deficiency
D. Liver failure
Thrombocytopenia
Which of the following is not a cause of thrombocytopenia?
A. Acute myeloid leukaemia
B. Vitamin K deficiency
C. Aplastic anaemia
D. ITP
Vit K deficiency does not.
AML, Aplastic Anaemia, ITP, may all cause thrombocytopenia.
This patient has spontaneous bruising and petechiae formation which platelet count is most likely?
A. 30
B. 10
C. 50
D. 100
10