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Studies in a Case of Bernard-Soulier Syndrome
Acta Haematologica, 2009In two siblings, brother and sister, with a history of bleeding Bernard-Soulier syndrome was diagnosed: The stronger affected sister showed a high percentage of giant blood platelets, an abnormal platelet spreading test and a defect in ristocetin and bovine fibrinogen-induced platelet aggregation.
W D, Zwierzina +5 more
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Ultrastructure of Platelets in Bernard-Soulier Syndrome
Mayo Clinic Proceedings, 1975The platelets of a patient with the Bernard-Soulier syndrome were studied by electron microscopy. The main abnormalities were the presence of giant and often round platelets, hypertrophic and frequently widely dilated open canalicular system, disorganized microtubules, and platelets with sparse or absent granulation.
J E, Maldonado +3 more
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Baillière's Clinical Haematology, 1989
Summary Bernard-Soulier syndrome (BSS) is a rare autosomal bleeding disorder characterized clinically by prolonged skin bleeding time, normal clot retraction and thrombocytopenia with large and morphologically abnormal platelets, and biochemically by the absence of platelet membrane glycoproteins (GP)Ib, V and IX. GP Ib and GPIX exist in the platelet
Michael C. Berndt +2 more
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Summary Bernard-Soulier syndrome (BSS) is a rare autosomal bleeding disorder characterized clinically by prolonged skin bleeding time, normal clot retraction and thrombocytopenia with large and morphologically abnormal platelets, and biochemically by the absence of platelet membrane glycoproteins (GP)Ib, V and IX. GP Ib and GPIX exist in the platelet
Michael C. Berndt +2 more
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2014
Bernard Soulier Syndrome is an autosomal recessive inherited platelet function disorder characterized by mild thrombocytopenia abnormally large platelets on peripheral blood smear It is clinically manifested by epistaxis and purpura ranging from mild to moderate degree In this article an 8 year old case with Bernard Soulier syndrome diagnosed after ...
CEVİT, Ömer +5 more
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Bernard Soulier Syndrome is an autosomal recessive inherited platelet function disorder characterized by mild thrombocytopenia abnormally large platelets on peripheral blood smear It is clinically manifested by epistaxis and purpura ranging from mild to moderate degree In this article an 8 year old case with Bernard Soulier syndrome diagnosed after ...
CEVİT, Ömer +5 more
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Bernard-Soulier syndrome and pregnancy: a case report
Annales de biologie clinique, 2015Bernard-Soulier syndrome is an inherited bleeding disorder. Due to the rarity of the combination of this syndrome and pregnancy, data on the clinical course and outcome of pregnancy in women with Bernard-Soulier syndrome is scattered in individual case reports and there is no consensus in the management of SBS.
Mohamed, Smaoui +6 more
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Bailliere's clinical haematology, 1989
Bernard-Soulier syndrome (BSS) is a rare autosomal bleeding disorder characterized clinically by prolonged skin bleeding time, normal clot retraction and thrombocytopenia with large and morphologically abnormal platelets, and biochemically by the absence of platelet membrane glycoproteins (GP) Ib, V and IX.
M C, Berndt +2 more
openaire +1 more source
Bernard-Soulier syndrome (BSS) is a rare autosomal bleeding disorder characterized clinically by prolonged skin bleeding time, normal clot retraction and thrombocytopenia with large and morphologically abnormal platelets, and biochemically by the absence of platelet membrane glycoproteins (GP) Ib, V and IX.
M C, Berndt +2 more
openaire +1 more source
Studies on the megakaryocytes of a patient with the Bernard‐Soulier syndrome
British Journal of Haematology, 1990Summary We have used monoclonal antibodies AP‐1 (anti‐GP Ibα), AP‐2 (anti‐GP IIb‐IIIa) and FMC 25 (anti‐GP IX) in immunofluorescence and immunocytochemical studies on megakaryocytes (MK) isolated from a Bernard‐Soulier syndrome (BSS) patient whose giant platelets were characteristically deficient in GP Ib‐IX complexes.
P, Hourdillé +5 more
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Bernard–Soulier Syndrome in pregnancy: case report and review of the literature
International Journal of Laboratory Hematology, 1998Muhieddine Seoud, Ali Shamseddine
exaly