Case 26 – update 2

Thanks for your comments so far. We have established that our patient is a 38 year old man who is due to go to theatre today. He has an isolated prolongation of his APTT (at 47 secs) and had a similar result a year ago. You also know that he had a major orthopaedic procedure some years ago, but this was in South Africa, so you have no access to blood results from  this period. Apparently he does not recall any particular issues with bleeding at this point.

You were all keen to establish whether this abnormal APTT is due to a clinically less relevant inhibitor such as a lupus anticoagulant, or a clinically concerning inhibitor, such as a an anti-factor VIII antibody which can cause acquired haemophilia. To determine this you would have done a DRVVT (lupus anticoagulant investigation for the non-lab readers) or mixing studies with attention to parallelism. There were also plenty of requests for testing the intrinsic factors (i.e. VIII, IX, XI and XII).

The clinicians might have been less sure about the tests needed but many were shouting for a proper bleeding history, including a family history. You pointed out that an acquired haemophilia would be vanishingly rare in a 38 year old patient, which is a very reasonable point, and that acquired haemophilia patients usually present with severe bleeding, which is not the case with this patient. A number of people suggested a FXII deficiency may be the issue as the patient apparently has no bleeding problems.

So, what happens next?

The lab staff alert you (as the haematologist) to the fact that the patient is listed for theatre today. You ask them to do the intrinsic factors (of course they had already planned that anyway…) whilst getting on the phone to the surgeons to alert them to your concerns.

You find out he is due to have an elective procedure to remove some lumbar osteophytes which have been causing him back pain. He has no significant past medical history. As regards bleeding history he has had major surgery including pinning of his femur after a car accident in South Africa aged 20, which didn’t cause any real bleeding problems. He does recall having some prolonged blood loss after a dental extraction but never needed stitches or other intervention. He doesn’t think anyone else in his family has a bleeding disorder.

He is cancelled from today’s list and you’re pleased he was when you see his factor assay results:

FVIII: 17%

FIX: 87%

FXI: 98%

FXII: 88%

QUESTIONS:

Are you surprised by the lack of bleeding history?

If so can you explain it?

If not, can you tell us why?

How would you plan for this patient’s rescheduled surgery?

Let us know your thoughts over on twitter and PLEASE PLEASE PLEASE use the hashtag #teamhaem in your answers so everyone else can see your clever comments! Comment is free so tell us what your thoughts are on this case.

The case will continue to evolve so please keep checking back.

All our cases are fictional to ensure patient confidentiality.

Teamhaem are not in a position of authority; rather we hope to inspire thought and debate about haematology via social media.

RESOURCES

For the non-BMSs the laboratory tests may be pretty unfamiliar. For those who are interested in understanding and learning more you can’t find a better resource than: www.practical-haemostasis.com.

About TeamHaem

Online education and discussion about all things haematological
This entry was posted in Inherited bleeding and tagged , , . Bookmark the permalink.

One Response to Case 26 – update 2

  1. David Gurney says:

    Notably missing is vWF testing. Possible mild Haemophilia A but surprised he didn’t bleed during surgery. Cover with tranexamic acid or DDAVP?

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s