Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2021.09.021
Brazilian Journal of Anesthesiology
Case Report

Perioperative administration of recombinant activated factor VII in a Glanzmann's thrombasthenia patient with platelet refractoriness: case report

Flora Margarida Barra Bisinotto, Laura Bisinotto Martins, Giovanini Pires de Camargos, Marcelo de Paula Bianco

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Abstract

Glanzmann's Trombasthenia (GT) is a genetic disorder, that develops with a tendency toward bleeding and is characterized by the absence or decrease in platelet aggregation. Surgical bleeding may be difficult to control. Platelet transfusion is the main treatment, albeit refractoriness can occur. We describe the case of a patient with GT and platelet refractoriness, who was submitted to radical prostatectomy and dental extraction. The perioperative treatment with apheresis platelet concentrate and activated recombinant factor seven allowed the procedures to be performed uneventfully. We discuss the complexity of the case and the treatment option.

Keywords

Surgery;  Prostatectomy;  Dental extraction;  Disorders;  Hematological;  Activated recombinant factor VII

References

1 T Solh, A Botsford, M. Solh Glanzmann's thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options J Blood Med, 6 (2015), pp. 219-227

2 MC Poon, G Di Minno, R D'Oiron, et al. New insights into the treatment of Glanzmann thrombasthenia Transfus Med Rev, 30 (2016), pp. 92-99

3 MC Poon, R D'Oiron Alloimmunization in congenital deficiencies of platelet surface glycoproteins: focus on Glanzmann's thrombasthenia and BernardSoulier's Syndrome Semin Thromb Hemost, 44 (2018), pp. 604-614

4 EN Duman, S Saylan, B. Cekic Conduta no perioperatório de paciente pediátrico com trombastenia de Glanzmann durante adenoidectomia Rev Bras Anestesiol, 62 (2012), pp. 548-553

5 MC. Poon The use of recombinant activated factor VII in patients with Glanzmann's thrombasthenia Thromb Haemost, 121 (2021), pp. 332-340

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Braz J Anesthesiol

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