Treatment of a patient with Bernard-Soulier syndrome and recurrent nosebleeds with recombinant factor VIIa

M Peters, H Heijboer - Thrombosis and haemostasis, 1998 - thieme-connect.com
Treatment of a Patient with Bernard-Soulier Syndrome and Recurrent Nosebleeds with
Recombinant VIIa Page 1 352 Letters to the Editor Dear Sir, Bernard-Soulier syndrome (BSS) is
a rare congenital bleeding disorder characterised by a prolonged bleeding time, large platelets
and thrombocytopenia. Aggregation of BSS platelets is normal in response to ADP, epinephrine,
collagen and arachidonic acid but there is no agglutination in response to ristocetin (1). The
disorder is due to absence or abnormality of the platelet membrane glycoprotein complex Ib-IX-V …