Article
Patients with Bernard-Soulier syndrome and different severity of the bleeding phenotype.
Blood cells, molecules & diseases - 1 Sept 2017
Boeckelmann D, Hengartner H, Greinacher A, Nowak-Göttl U, Sachs U J, Peter K, Sandrock-Lang K, Zieger B
Abstract excerpt
Bernard-Soulier syndrome is a rare (1:1million), hereditary bleeding disorder caused by defects of the platelet GPIb-IX-V complex. Patients suffer from mucocutaneous bleedings. Typical are thrombocytopenia, giant platelets and impaired agglutination after stimulation with ristocetin. In populations in which consanguineous marriages are common the frequency of the disorder is increased because Bernard-Soulier...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.