A lack of consistency exists surrounding the management of intracranial hemorrhage (ICH) in patients with von Willebrand disease (VWD), according to a clinical case report and literature review.
“Intracranial hemorrhage (ICH) in von Willebrand patients is a very rare event,” Ezio Zanon, MD, of the University Hospital of Padua (Italy), and his colleagues wrote in a letter to the editor published in.
The researchers reported findings from a case of a woman with type 2B VWD who experienced two spontaneous ICH events 12 years apart. Shortly after her admission for the second episode, the patient died as a result of severe bleeding.
Additionally, the team reviewed the body of literature for other cases of ICH and summarized the management strategies used in these patients.
“To date, only a few cases of intracranial hemorrhages in VWD are reported in literature,” the researchers wrote.
After analysis, the researchers found that a lack of consistency exists in the management of ICH in patients with VWD.
With respect to management strategies, Dr. Zanon and his colleagues suggested that ongoing prophylactic therapy with replacement concentrate could lower the risk of ICH recurrence. In the case presented, the patient was started on prophylaxis after the first ICH episode but it was interrupted 1 year later.
The team also recommended that hypertension should be closely monitored as it is a key risk factor for developing ICH. In the case presented, the patient had poorly controlled arterial hypertension.
“Studies and registries would be very important to define the burden of ICH in VWD patients clearly and establish its proper management,” they wrote.
No funding sources were reported. The authors reported having no conflicts of interest.
SOURCE: Zanon E et al. Haemophilia. 2019 Mar 29. .