The main aim of this case was to remember that the coagulation screen alone is not suitable for ruling out a bleeding disorder. The most common inherited bleeding disorder frequently will have a normal clotting screens. Indiscriminate clotting screens are not appropriate without prior knowledge of the bleeding history. This is especially true in pre-operative assessment clinics, where they may falsy reassure the clinician that there is no bleeding risk. Likewise they may cause alarm/delay/needing further/increased cost when results are abnormal, when after further investigations results are subsequently normal or a factor XII deficiency or lupus anticoagulant is found (no increased bleeding with these).
A bleeding history should be performed prior to major procedures. See http://www.wfh.org/en/resources/bleeding-assessment-tool-condensed-mcmdm-1-vwd for a structures bleeding questionnaire validated for the use in diagnosing von Willebrand disease (but also useful to help with structuring a bleeding history). Family history is also important to identify an inherited condition.
Bleeding disorders with normal coagulation screens include:
- von Willebrand’s disease
- Platelet function disorders
- Alpha-2 anti-plasmin deficiency
- Factor XIII deficiency
- Mild factor deficiencies (not severe enough to prolong the APTT)
Von Willebrand disease is diagnosed using a number of tests. The main two are von Willebrand antigen (looking at the amount) and von Willebrand ristocetin cofactor and/or collagen binding (looking at function). Von Willebrand disease is classified as:
- Type I – reduction in amount of vWF
- Type II – problem with function of vWF +/- amount
- Type III – near/complete absence of vWF
Our patient had type I vWD as the vWF-RicoF:vWF-Ag was >0.6
vWD is treated with a combination of:
- Tranexamic acid
- DDAVP – patients should usually have a diagnostic DDAVP challenge prior to use (often only works well in type I and may not give sustained therapeutic levels)
- von Willebrand factor concentrate
- OCP and other hormonal therapies for menorrhagia
- Guidance on the assessment of bleeding risk prior to surgery or invasive procedures
- NICE guidance: routine preoperative tests for elective surgery
- Guidance for diagnosis and management of von Willebrand disease
- Bleeding questionnaire
Please reply to us (@TeamHaem) on Twitter and always include #TeamHaem to allow others to follow your comments. Please join in the debate and learn about haematological problems along the way. The case will continue to evolve over the coming week so keep checking #TeamHaem on Twitter for more information.
Please note – all cases on TeamHaem are entirely fictional to protect patient confidentiality.
TeamHaem are not a position of authority. It is an educational platform to allow discussion and learning.