I have dated when I wrote questions, to help you gauge if particular answers may be obsolete.

Question 1 (2018)

Give a differential diagnosis for a FVIII:C of 0.15 iu/ml

Question 2 (2018)

A. A pregnant woman tells you she has a family history of ‘haemophilia’. How would you proceed

B. You find nothing from the above, what possibilities remain?

Question 3 (2018)

A. In an asymptomatic woman, whose father has severe haemophila A and mother is normal, what is the risk that her daughter is a carrier of severe haemophilia A?

B. The daughter is a carrier (FVIII 0.68 iu/ml). She goes on to have two twin girls of her own. One twin has a FVIII of 0.69 iu/ml. What would you predict the levels to be in the other twin?
  

Question 4 (2018)

In which situations can you be sure someone is an obligate carrier of haemophilia A?

Question 5 (2021)

An 85 year old woman presents to the emergency department with epistaxis. She is confused and disorientated. She is known to take apixaban for atrial fibrillation. Collateral history suggests the confusion is new and there is no past history of bleeding/bruising.

The clotting screen is as follows:

  • Prothrombin time: 14.6 seconds (NR 10-13)

  • Activated partial thromboplastin time (APTT): 180 seconds (NR 24-38)

  • Thrombin time: 14 seconds (NR10-16)

  • Clauss Fibrinogen: 5.3 g/l (NR 2-4)

A. List the differential diagnosis for these clotting results in isolation

 

Further testing is performed (samples treated with DOAC-Remove prior to testing):

  • APTT 50/50 mix: 60 seconds

  • Lupus insensitive APTT: 92 seconds

  • Dilute Russell Viper Venom Time (dRVVT) ratio: 1.25 (NR 0-1.19)

  • Dilute Russell Viper Venom Time correction: 16% (NR <10%)

  • Apixaban assay: 106ng/ml (Peak level NR 91-320)

B. Describe the principle of the dRVVT test for lupus anticoagulant

Further coagulation studies are performed (samples treated with DOAC-Remove prior to testing):

  • Factor II: 1.11 IU/ml

  • Factor V: 1.07 IU/ml

  • Factor VIII: <0.01 IU/ml

  • Factor IX: 0.93 IU/ml

  • Factor XI: 0.70 IU/ml

  • Factor XII: 0.45 IU/ml

  • Time dependent inhibitor screen immediate 50:50 mix 67.2 seconds

  • Time dependent inhibitor screen 2 hour 50:50 mix 131.2 seconds

C. State the final diagnosis in this case and briefly summarise your initial management of this patient

question 6 (2024)

Mr Cobblepot is a 65 year old man admitted for an elective right total knee replacement. He has a fall post-operatively which delays his rehabilitation and as a result he is making good progress but remains an inpatient seven days after the procedure. On day 8 post-op he reports a painful, swollen, red left leg.

Current medications: Lisinopril, Atorvastatin, Paracetamol, Oxycodone liquid, Prophylactic Tinzaparin

Bloods pre-op:                  Hb 135, WBC 5.5, Platelets 230, Creatinine 60, ALT 10, Bili 5

Bloods on day 8:               Hb 115, WBC 11, Platelets 45, Creatinine 65, ALT 11, Bili 6, CRP 30

 

a) What test(s) would you perform next?

b) Briefly describe the principles of one laboratory assay, of your choice, that can be used to confirm the suspected cause of thrombocytopenia

c) Recommend a management plan 

d) Three years later, the patient requires cardiac bypass surgery. The cardiothoracic team would prefer to use unfractionated heparin to support the surgery. They request your advice regarding his suitability to receive this.