Surgical treatment of ruptured ovarian cyst in a patient with Bernard-Soulier syndrome

Authors

DOI:

https://doi.org/10.33448/rsd-v11i2.25366

Keywords:

Ovarian Cysts; Bernard-Soulier Syndrome; Abdomen acute.

Abstract

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.

References

Afrasiabi, A. et al. (2007). Genetic characterization of patients with Bernard-Soulier syndrome and their relatives from Southern Iran. Platelets. 18(6), 409-13.

Afshar-Kaharghan, V., & López, J. A. (1997) Bernard-Soulier Syndrome Caused by a Dinucleotide Deletion and Reading Frameshift in the Region Encoding the Glycoprotein Ibα Transmembrane Domain. Blood. 90(7), 2634-43.

Almeida, A. M., Khair, K., Hann, I., & Liesner, R. (2003). The use of recombinant factor VIIa in children with inherited platelet function disorders. British Journal Of Haematology. 121(3), 477-81. http://dx.doi.org/10.1046/j.1365-2141.2003.04286.x.

Almomani, M. H., & Mangla, A. (2021) Bernard Soulier Syndrome. Statpearls. 1(1), 1-8.

Andrews, R., & Berndt, M. (2013). Bernard-Soulier Syndrome: an update. Seminars In Thrombosis And Hemostasis. 39(6), 656-62. http://dx.doi.org/10.1055/s-0033-1353390.

Araújo, S., Alves, W., Bueno, L. M., Dionísio, C., & Siqueira, P. H. (2002). Epistaxe como manifestação frequente da síndrome de Bernard-Soulier. Revista Brasileira de Otorrinolaringologia. 68(2), 284-86. http://dx.doi.org/10.1590/s0034-72992002000200021.

Berndt, M. C., & Andrews, R. K. (2011). Bernard-Soulier syndrome. Haematologica. 96(3), 355-59. http://dx.doi.org/10.3324/haematol.2010.039883.

Bhadra, D., & Chakraborty S. (2020). Bernard-Soulier syndrome (BSS) with uncontrollable menorrhagia. Asian J Transfus Sci. 14(1), 93-5. https://www.ajts.org/article.asp?issn=0973-6247;year=2020;volume=14;issue=1;spage=93;epage=95;aulast=Bhadra.

Boeckelmann, D, Hengartner, H., Greinacher, A., Nowak-Göttl, U., Sachs, U., Peter, K., Sandrock-lang, K., & Zieger, B. (2017). Patients with Bernard-Soulier syndrome and different severity of the bleeding phenotype. Blood Cells, Molecules, And Diseases. 67(1), 69-74. http://dx.doi.org/10.1016/j.bcmd.2017.01.010.

Farhan, S., & Iqbal I, A. N. (2019). Bernard Soulier Syndrome: 10 years' experience at a tertiary care hospital. Pak J Med Sci. 35(3), 705-08. 10.12669/pjms.35.3.980. PMID: 31258580; PMCID: PMC6572988. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6572988/.

Feitosa Júnior, V. N. (2018). Abordagem do abdome agudo ginecológico: trabalho de conclusão de residência médica (residência médica em cirurgia geral). Hospital Geral de Fortaleza, http://extranet.hgf.ce.gov.br/jspui/bitstream/123456789/358/1/2018_TCR_CirurgiaGeral_FeitosaJ%c3%banior_VAL.pdf.

Freitas-Dias, R. (2017). Metodologia Científica: um guia prático para profissionais da saúde. Editor: Ricardo Freitas

Giuliano, L.C., Luciano, L.S., Portilho, S. S., Waldrich, L., Arruda, J. F., & São Tiago, L. E. (2020). Diagnóstico ocasional de cisto ovariano durante procedimento de oclusão percutânea de comunicação interatrial. J Transcat Intervent. https://doi.org/10.31160/JOTCI202028A20200023.

Graigner, J. D., Thachil, J., & Will, A. M. (2018). How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults. British Journal Of Haematology. 182(5), 621-32. http://dx.doi.org/10.1111/bjh.15409.

Greinacher, A. et al. (1993). Evidence that DDAVP transiently improves hemostasis in Bernard-Soulier syndrome independent of von Willebrand-Factor. Annals Of Hematology. 67(1), 149-51.

Gupta, A. et al. (2020). A case report of Bernard Soulier Syndrome and its management in minor oral surgical procedures. International Journal Of Current. 9(12), 13545-48.

Kunishima, S., Kamiya, T., & Saito, H. (2002). Genetic Abnormalities of Bernard-Soulier Syndrome. International Journal Of Hematology. 76(4), 319-27. http://dx.doi.org/10.1007/bf02982690.

Lanza, F. (2006). Bernard-Soulier syndrome (Hemorrhagiparous thrombocytic dystrophy). Orphanet Journal Of Rare Diseases. 1(1), 1-6. http://dx.doi.org/10.1186/1750-1172-1-46.

Ozelo, M. C., Svirin, P., & Larina, L. (2005). Use of recombinant factor VIIa in the management of severe bleeding episodes in patients with Bernard–Soulier syndrome. Annals Of Hematology. 84(12), 816-22. http://dx.doi.org/10.1007/s00277-005-1080-y.

Reisi, N. (2020). Bernard-Soulier syndrome or idiopathic thrombocytopenic purpura: A case series. Caspian J Intern Med. 11(1), 105-09. 10.22088/cjim.11.1.105. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6992729/.

Savoia, A., Kunishima, S., Rocco, D. de., Zieger, B., Rand, M. L., Pujol-Moix, N., Caliskan, U., Tokgoz, H., Pecci, A., & Noris, P. (2014). Spectrum of the Mutations in Bernard-Soulier Syndrome. Human Mutation. 35(9), 1033-45. http://dx.doi.org/10.1002/humu.22607.

Published

03/02/2022

How to Cite

PIRES, J. V. A.; SANTANA, J. F. C. L. de .; FERNANDES, E. de B. D.; BERTON, N. C.; FRANCHELLO, I. F.; SANTOS, R. M.; MARQUES, C. P. .; FRANCISCO, L. C. .; LARA, R. F. . Surgical treatment of ruptured ovarian cyst in a patient with Bernard-Soulier syndrome. Research, Society and Development, [S. l.], v. 11, n. 2, p. e47911225366, 2022. DOI: 10.33448/rsd-v11i2.25366. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/25366. Acesso em: 22 dec. 2024.

Issue

Section

Health Sciences