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[Bernard-Soulier thrombopathy: a diagnostic pitfall]
Kadoudja Chemlal1, Marie-José Grange, Marie-Geneviève Huisse
1Service de Médecine Interne A, Hôpital Max-Fourestier, 403, avenue de la République, 92000 Nanterre, France. khadoudja.chemlal@CH-nanterre.fr
First described in 1948, Bernard-Soulier syndrome is an uncommon hereditary thrombopathy characterized by abnormal expression of the GPIb-IX-V complex which inhibits platelet migration to the site of endothelial trauma. Our case illustrates the pathophysiological mechanisms involved and points out the similarity with idiopathic thrombopenic purpura.
First described in 1948, Bernard-Soulier syndrome is an uncommon hereditary thrombopathy characterized by abnormal expression of the GPIb-IX-V complex which inhibits platelet migration to the site of endothelial trauma. Our case illustrates the pathophysiological mechanisms involved and points out the similarity with idiopathic thrombopenic purpura.