Von Willebrand disease within the collective of haemophilic patients as reason for unexpected bleeding episodes.
In the treatment of haemophilia A and B, recombinant coagulation factors are increasingly replacing plasma-derived factor VIII and IX concentrates. Although provided with replacement therapy, individual patients may exhibit bleeding episodes, which are difficult to control. These bleeds may be caused by von Willebrand disease (VWD) as an additional underlying coagulation disorder. We report in the present study our experience that in the collective of haemophilic patients, VWD must be anticipated at least with the same order of magnitude as it appears in a normal healthy population. Among the patients at our treatment centre, two patients (1.5%) were identified as suffering from VWD in addition to haemophilia A. In the collective of haemophilia B patients at our centre, three patients (10%) with VWD were found. Two of these patients exhibited unexpected severe bleeding episodes, which could only satisfactorily be controlled by the administration of Haemate-P or DDAVP supplementary to the recombinant coagulation factor concentrate.
Siegmund
B
B
Institut für Thrombophilie und Hämostaseologie, Munster, Germany. ith@haemophilie-zentrum.de
Richter
H
H
Pollmann
H
H
D001780:Blood Coagulation Tests; D006467:Hemophilia A / Q000097:blood / Q000150:complications*; D002836:Hemophilia B / Q000097:blood / Q000150:complications*; D006470:Hemorrhage / Q000097:blood / Q000209:etiology*; D006801:Humans; D011446:Prospective Studies; D014842:von Willebrand Diseases / Q000097:blood / Q000150:complications*