Clinical and biological studies in the Bernard-Soulier syndrome
Tobelem, G.
Nouvelle Revue Francaise d'Hematologie 16(3): 407-411
1976
ISSN/ISBN: 0029-4810 PMID: 1087721 Document Number: 108833
Bernard-Soulier syndrome is a rare constitutional thrombopathy, the main clinical feature is a bleeding tendency which is variable. The long bleeding time, in spite of a normal or little decreased platelet count, has been recently explained: the giant platelets have a defective adhesion to subendothelium. This abnormal adhesion could be related with an abnormal pattern of platelet membrane glycoproteins. Interactions between vessel wall, von Willebrand's factor and platelets are discussed.