Treatment for Bernard-Soulier Syndrome
For severe bleeding, people with BSS may be given platelets from a blood donor. A problem can happen because the donor’s platelets are normal and have GPIb/IX. The platelets of the person with BSS do not. This difference can cause the immune system of the person with BSS to make antibodies against the donated platelets. If this occurs, future transfusions with platelets may not work because the antibodies will quickly destroy the donated platelets. To help prevent this, whenever possible people with BSS should only get platelets that have been filtered to remove white blood cells (leukocytes) and that have been HLA matched.
Recombinant factor VIIa (NovoSeven®) has been used to treat severe bleeding in people with BSS.