Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1590/S0034-70942012000400009
Brazilian Journal of Anesthesiology
Clinical Information

Conduta no perioperatório de paciente pediátrico com trombastenia de glanzmann durante adenoidectomia

Perioperative management of a pediatric patient with glanzmann's thrombasthenia during adenoidectomy

Erdem Nail Duman; Sedat Saylan; Bahanur Cekic

http://dx.doi.org/10.1590/S0034-70942012000400009 Braz J Anesthesiol, vol.62, n4, p.551-553, 2012
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Resumo

JUSTIFICATIVA E OBJETIVOS: Trombastenia de Glanzmann (TG) é uma doença autossômica recessivamente hereditária das plaquetas. Não há nenhum tratamento específico. A transfusão de plaquetas é atualmente o tratamento padrão quando o sangramento não responde a medidas locais e/ou a medicamentos antifibrinolíticos, podendo, entretanto, resultar em aloimunização. O fator VII recombinante ativado (rFVIIa) pode ser usado para evitar a transfusão recorrente de plaquetas. RELATO DE CASO: Apresentamos um tratamento precoce com dose baixa de rFVIIa associada à transfusão de plaquetas em um caso pediátrico (cinco anos de idade), com diagnóstico de TG e apresentando sangramento prolongado durante adenoidectomia eletiva. Uma dose total de 1.200 mg (60 µg.kg-1) de rFVIIa obteve sucesso em estancar o sangramento, o que pode ser aceito como uma dose baixa. CONCLUSÕES: Relatos de casos podem encorajar o uso de tratamento precoce com baixas doses de rFVIIa em hemorragias graves que não estacam a despeito da transfusão de plaquetas e na prevenção de sangramento em procedimentos cirúrgicos em pacientes com TG. Estudos adicionais são necessários para definir a dose mínima eficaz. Portanto, as tentativas para determinar a dose eficaz mais baixa desse composto devem ser incentivadas consideando o resultado deste caso em face de restrições financeiras no sistema de saúde.

Palavras-chave

CIRURGIA, Otorrionolaringológica, DOENÇAS, Hematológica, Fator VII Ativado

Abstract

BACKGROUND AND OBJECTIVE: Glanzmann's thrombasthenia (GT) is an autosomal recessively inherited platelet disorder. There is not any specific treatment. Platelet transfusion is currently the standard treatment when bleeding does not respond to local measures and/or antifibrinolytic treatment, although it may result in alloimmunization. Recombinant activated factor VII (rFVIIa) might be used to avoid recurrent platelet transfusion. CASE REPORT: We present early treatment with low-dose rFVIIa additional to platelet transfusion in a 5-year-old pediatric case with diagnosis of GT who developed prolonged bleeding under an elective adenoidectomy surgery. A total dose of 1,200 µg (60 µg.kg-1) rFVIIa could successfully stop bleeding, what can be accepted as low dose usage. CONCLUSIONS: Such case reports may encourage the use of early treatment with low doses of rFVIIa in severe bleeds that did not stop despite of platelet transfusion, as well as in preventing bleeding in surgical procedures in patients with GT. Actually, additional studies are needed to define the minimal effective dose and attempts to determine the lowest effective dose may be encouraged by the result of this case, considering financial restrictions in the health care system.

Keywords

Anesthesia, General, Adenoidectomy, Factor VIIa, Thrombasthenia

References

Nair S, Ghosh K, Kulkarni B. Glanzmann's thrombasthenia: updated. Platelets. 2002;13:387-393.

Poon M-C, d'Oiron R, von Depka M. Prophylactic and therapeutic recombinant factor VIIa administration to patients with Glanzmann's thrombasthenia: results of an international survey. J Thromb Haemost. 2004;2:1096-1103.

Erduran E, Aksoy A, Zaman D. The use of recombinant FVIIa in a patient with Glanzmann thrombasthenia with uncontrolled bleeding after tonsillectomy. Blood Coagul Fibrinolysis. 2009;20(3):215-217.

Poon M-C, Demers C, Jobin F. Recombinant factor VIIa is effective for bleeding and surgery in patients with Glanzmann thrombasthenia. Blood. 1999;94(11):3951-3953.

Almeida AM, Khair K, Hann I. The use of recombinant factor VIIa in children with inherited platelet function disorders. Br J Haematol. 2003;121(3):477-481.

O'Connell NM, Perry DJ, Hodgson AJ. Recombinant FVIIa in the management of uncontrolled hemorrhage. Transfusion. 2003;43(12):1711-1716.

Tengborn L, Petruson B. A patient with Glanzmann thrombasthenia and epistaxis successfully treated with recombinant factor VIIa. Thromb Haemost. 1996;5(6):981-982.

Uzunlar HI, Eroglu A, Senel AC. A patient with Glanzmann's thrombasthenia for emergent abdominal surgery. Anesth Analg. 2004;99:1258-1260.

Coppola A, Tufano A, Cimino E. Recombinant factor VIIa in a patient with Glanzmann's thrombasthenia undergoing gynecological surgery: open issues in light of successful treatment. Thromb Haemost. 2004;92:1450-1452.

. .

De Gasperi A. Intraoperative use of recombinant activated factor VII (rFVIIa). Minerva Anestesiol. 2006;72(6):489-494.

Chiara O, Cimbanassi S, Brioschi PR. Treatment of critical bleeding in trauma patients. Minerva Anestesiol. 2006;72(6):383-387.

Marson F, Farnia A, Callegher L. Use of recombinant activated factor VII (rFVIIa-NovoSeven) in the treatment of uncontrolled postsurgical hemorrhage in a patient with deep venous thrombosis and caval filter: A case report. Minerva Anestesiol. 2006;72(7-8):675-682.

Cozzi P, Chiumello D, Tubiolo D. Total hepatectomy, recombinant activated factor VII and rescue liver transplantation. Minerva Anestesiol. 2010;76(7):550-553.

Lusher JM. Early treatment with recombinant factor VIIa results in greater efficacy with less product. Eur J Haematol. 1998;63(^sSuppl):7-10.

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