Perioperative considerations in a girl with Glanzmann thrombasthenia. Case report
Introduction: Glanzmann thromboasthenia is a rare congenital bleeding disorder caused by a mutation in platelet glycoprotein α-IIb and β3 encoding genes (ITGA2B; 607759 and ITGB3; 173470) in chromosomes 17q21.31 and 17q21.32 , respectively, which results in a qualitative or quantitative alteration of the platelet integrin αIIbβ3 (glycoprotein IIb/IIIa) receptor. Glanzmann thromboasthenia is classified as type I when less than 5% of glycoprotein αIIbβ3 is expressed, and as type II when more than 5% is expressed.
Case presentation: Description of the perioperative management of a 13-year-old female patient with Glanzmann thromboasthenia who underwent endoscopic anterior bilateral ethmoidectomy. Management was centered on prophylactic platelet transfusion plus the use of tranexamic acid, as well as thromboelastographic determination of hemostasis. There were no bleeding complications during or after the procedure.
Conclusions: Pediatric patients with Glanzmann thromboasthenia are at a high risk of perioperastive bleeding. Platelet transfusion is the best prophylactic and therapeutic alternative; however, even in the absence of anti-platelet antibodies, it may not be effective, and viscoelastic testing must be used for assessment during the surgical procedure in order to improve patient safety.
Solh T, Botsford A, Solh M. Glanzmann’s thrombasthenia: pathogenesis, diagnosis, and current and emerging treatment options. J Blood Med. 2015;6:219-27.
Barg AA, Hauschner H, Misgav M, Lubetsky A, Levy-mendelowitz S, Livnat T, et al. A novel approach using ancillary tests to guide treatment of Glanzmann thrombasthenia patients undergoing surgical procedures. Blood Cells, Mol Dis. 2018;72:44-8. https://doi.org/10.1016/j.bcmd.2018.07.003
Franchini M, Lippi G. NovoSeven (recombinant factor VIIa ) for the treatment of bleeding episodes and perioperative management in patients with Glanzmann ’ s thrombasthenia. Expert Rev Hematol. 2014;733-40.
Poon M, Oiron R, Zotz RB, Bindslev N, Nicola M, Di D, et al. The international , prospective Glanzmann Thrombasthenia registry: treatment and outcomes in surgical intervention.Coagulation & Its Disorders. 2015(100):1038-44.
Rajpurkar M, Chitlur M, Recht M, Cooper DL. Use of recombinant activated factor VII in patients with Glanzmann’s thrombasthenia: a review of the literature. Hemophilia. 2014;464-71.
Poon M, Di G, Zotz R. New Insights into the treatment of Glanzmann thrombasthenia. Transfus Med Rev. 2016;30(2):92-9. http://dx.doi.org/10.1016/j.tmrv.2016.01.001
Al-Battat S, Rand ML, Bouskill V, et al. Glanzmann thrombasthenia platelets compete with transfused platelets, reducing the haemostatic impact of platelet transfusions. Br J Haematol. 2018;181(3):410‐3. doi:10.1111/bjh.14623
Fiore M, D´Oiron R, Pillois X. Anti-a IIb b 3 immunization in Glanzmann thrombasthenia : review of literature and treatment recommendations. Br J Haematol. 2018;181(2):1-10.
Grassetto A, Fullin G, Lazzari F, et al. Perioperative ROTEM and ROTEMplatelet monitoring in a case of Glanzmann's thrombasthenia. Blood Coagul Fibrinolysis. 2017;28(1):96‐99. doi:10.1097/MBC.0000000000000532
Budnik I, Shenkman B, Morozova O, Andreichyn J, Einav Y. Correction of coagulopathy in thrombocytopenia and Glanzmann thrombasthenia models by fibrinogen and factor XIII as assessed by thromboelastometry. Pathophysiology. 2018;25(4):347‐51. doi:10.1016/j.pathophys.2018.05.005
Copyright (c) 2020 Sociedad Colombiana de Anestesiología y Reanimación (S.C.A.R.E.)
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.