Have you encountered such combination of rare bleeding disorders (RBD)?
We recently consulted female undergoing hysterectomy, diagnosed with vWF type I disease , with supposed antithrombin deficiency and factor XI deficiency.
What is your experience ? Is there possibility of such combination of RBDs?
Dear Peter,
sounds very interesting, and unlikely! However, in Biology, "never" does not exist...
Maybe the antithrombin deficiency was "leveled" out by the bleeding disorders...did the patient suffer from thromboembolic events? How old is she? I have seen only two patients with "real" antithrombin deficiency not caused by prolonged heparin usage, and both suffered from thromboembolic events before they turned 25! Both had residual activities of about 30% of antithombin. We managed one of the two when she was pregnant, and she did not have another thrombembolic event, and gave birth to a healthy child.
Personally, I have seen a couple of combinations of vWF disease and acquired FXIII deficiency in plastic surgery with large wound areas. We had a couple of FXI deficient patients during the last years, but none of those had any additional bleeding disorder except for iatrogenic by ASS or other drugs.
What we need is a good hematologist like you.
www.jmig.org/article/S1074-3804(05)80640-1/abstract