出版社：Grupo de Pesquisa Metodologias em Ensino e Aprendizagem em Ciências
摘要：Introduction: the Bernard-Soulier Syndrome (BSS) is a rare inherited bleeding disorder witch the prevalence is estimated at 1:1,000,000. Its abnormalities are due to defective expression of the glycoproteic complex GPIb/IX/V – the platelets receptor that is crucial for the establishment of the prime hemostasis. Objective: report a rare case of a ruptured ovarian cyst in a patient with Bernard-Soulier Síndromes. Methodology: it's a descriptive and transversal study that consists of the formulation of a case report, built from the analysis of a patient’s medical record with BSS, who evolved with ruptured ovarian cyst. For theoretical foundation we used articles indexed in PubMed's database written in portuguese and english. The following descriptors were considered: Ovarian Cysts, Bernard-Soulier Syndrome and Abdomen Acute. Conclusion: considering the frequency and severity of the hemorrhagic events as highly variable aspects among patients with BSS it is necessary that the surgeon always keep in mind the underlying hematological disorders when faced with a possible hemorrhagic acute abdomen.
