The document summarizes two cases seen by the regional haematology service. The first case involves a woman with von Willebrand disease who presented with shortness of breath and was found to have a respiratory infection rather than a pulmonary embolism. The second case involves a man with mild haemophilia A on long-term warfarin who presented with abdominal pain and was found to have multiple haematomas requiring factor VIII replacement and intensive care support. The document also provides background on managing inherited bleeding disorders in general hospitals and the role of specialized national coagulation centres.

1. Grand rounds MRHT
Coagulation disorders
Dr Gerard Crotty
Consultant haematologist
21 Oct 2020

2. Coagulation cascade

3. Coagulation cascade (simplified)
XII
XI
IX (VIII)
X (V)
VII
II
Fibrinogen Fibrin clot
Prothrombin time
Activated partial thromboplastin time

4. Midland Haematology Service
• Regional service – old MHB – three acute
hospitals MRHT, MRHM and MRHP
• 2 consultants – on call 1:2
• 2 registrars – on call 1:4 with the two medical
oncology registrars
• General haematology service
• National services include National Coagulation
Centre etc

5. Case 1
• 66 year old female presented MRHM ED
• Shortness of breath associated with chest
discomfort
• Tachypnoeic, tachycardic
• D dimers – 11067 ng/ml
• Lactate 4.9
• Troponin < 0.01
• Impression – outrule PE

6. Case 1 contd
• History of inherited bleeding disorder – von
Willebrand’s disease – noted by staff
• ED administered therapeutic LMWH and
referred medical
• Later call to haematology consultant
– who in turn called SJH NCC consultant
– vWD type 2 M
– Recommended Wilate 1500 u (F VIII/vWF) to
prevent bleeding

7. contd
• CTPA ASAP – no evidence of PE
• No further LMWH
• No further Wilate needed
• No bleeding
• Diagnosis – RTI ? Viral
– COVID-19 swab negative

8. Case 2
• 74 year old male present ED MRHT
• RUQ abdominal pain radiating to the chest
• 30 mins after eating fish and chips
• Nausea and diaphoresis
– PMH: hypertension, diabetes, atrial fibrillation,
obstructive sleep apnoea (on CPAP), type 2 diabetes,
CCF, pulmonary hypertension, dyslipidaemia,
Haemophila A (mild F VIII deficiency), diverticular
disease
– On warfarin

9. Case 2 - background
• 2016 – presented with new onset AF
• Discussed by medical team with his
coagulation haematologist – anticoagulation
advised against initially
• Later opted to go on warfarin – after review at
National Coagulation Centre and discussion of
the risk vs benefits
– Baseline VIII 10%
– CHADS VASC score 3

10. Severity of haemophilia

11. Contd
• Pain subsided, doing well
• US – fatty liver, mild intrahepatic bile duct
dilatation – MRCP to be considered
• MRCP – intraductal calculi
• ERCP planned (discussion with NCC re plan to do
this at SJH)
• Warfarin held
• Changed over to LMWH – INR 1.5
• Enoxaparin 1.5 mg sc daily

12. Contd
• ? Haematoma abd wall
• Enoxaparin held
• Deteriorated - with hypotension
• Initial diagnosis - sepsis
• Treated with piperacillin/tazobactam/fluids
• AKI
• CT abdo/pelvis planned
• Next morning – clinically jaundiced, abd mass, BP
100/65 – plan urgent CT and discuss with
haematology

13. contd
• BP 83/51
• Pale and clammy
• Urgent transfusion – O neg followed by cross matched
RBC
• On advice of NCC consultant – Factor VIII (Elocta) 5500
units (100% “rise”)
• Tranexamic acid
• Vitamin K (INR 1.3 so little residual warfarin effect)
• Platelets >100
• APTT ratio 1.2 after factor VIII
• Fibrinogen 5.5 g/L

14. contd
• CT – haematomas – pelvis, right rectus sheath
and left flank
• Further VIII dosing guided by levels done at
SJH
• ICU – noradrenaline inotropic support, further
RBC transfusion
• Next day – transferred ICU to ICU to SJH

15. Inherited bleeding disorders
• Mostly rare, generally single factor deficiencies
• Specialised national comprehensive centre
• National Haemophilia Council
• Product Selection Committee
• Comprehensive management
• Registered on database
• 24 hour access to database for diagnosis, baseline
level, other relevant issues eg inhibitors and
recommended treatment
• Phone us- we will phone the NCC consultant/SpR

16. SJH website

20. Management of inherited bleeding
disorders in a general hospital
• Patients attend the National Centre for review
• Patients on regular prophylaxis are on home treatment
• Typical interaction – patient presents with bleed –
consultation with centre – single dose of relevant
factor given and transferred for further management to
NCC
• Can haemophilia patients have thrombosis – yes.
Difficult issue to balance risk and benefit of
anticoagulation long term with/without coagulation
factor support
• With ageing population and longevity of severe
haemophilia patients will be an increasing issue

21. Inherited coagulation disorders

22. Coagulation cascade and screening tests
(may miss vWD and will miss factor XIII deficiency)
XII
XI
IX (VIII)
X (V)
VII
II
Fibrinogen Fibrin clot
Prothrombin time
Activated partial thromboplastin time

23. Types of von Willebrand disease

24. Conclusions
• Inherited coagulation disorders – seen rarely in general
hospital practice
• Most will already be known, diagnosed and registered
with access to diagnostic and therapeutic information
24/7 – always seek expert help immmediately
• Most coagulation disorders we see are acquired –
sepsis, DIC, liver disease, vitamin K deficiency, dilution
due to major haemorrhage/transfusion, acquired
inhibitors of coagulation factors (rare) and
anticoagulant drugs
• Thrombosis can occur in people with inherited
bleeding disorders will likely be an increasing issue