Thrombocytopenia Associated with Platelet Phagocytosis in Bernard-Soulier Syndrome (BSS)

BSS is defined as a prolonged bleeding time associated with giant platelets absence of ristocetin induced platelet aggregation related to the decreased platelet membrane glycoprotein I. Al though normal platelet count is usually observed during the first progressive thrombocytopenia is a commun evolution of the disease. A 11 year old boy with a BSS was examined twice; once in absence of bleeding: the main features of the disease were evaluated and allowed the diagnosis of BSS, the second time during a bleeding episod, the low platelet count was found to be associated with platelets phagocytosis by polymorphonuclears. This abnormal phagocytosis was studied in E.M. and beside the usual caracteristics of BSS platelets: large platelets, with membrane complexe formations, vacuolization and either absents of increased number of granules dispersed in the cytoplasm were observed around the polymorphonuclear white cells and inside the cells after phagocytosis. Platelet phagocytosis was reported in few cases of thrombocytopenia and occasionnally found to be associated with the “satellitism” phenomenon. This unic observation could provide an explanation for the decreased number of platelets usually observed in BSS.

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch

References and Relationships

• Bibliographic citation: Thrombocytopenia Associated with Platelet Phagocytosis in Bernard-Soulier Syndrome (BSS) ; volume:42 ; number:06 ; year:1979 ; Konferenz: VIIth International Congress on Thrombosis and Haemostasis; 1979-07-15; London ; Thrombosis and haemostasis ; 42, Heft 06 (1979)

Contributors, Places and Time

• Contributor: Jeanneau, C. ; Maisonneuve, P. ; Sultan, Y.

Further information

• DOI: 10.1055/s-0039-1687572
• URN: urn:nbn:de:101:1-2412190903301.436672656260
• Rights: Open Access unbekannt; Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 15.08.2025, 7:30 AM CEST