Jornal Vascular Brasileiro
https://www.jvascbras.org/article/doi/10.1590/1677-5449.200036
Jornal Vascular Brasileiro
Case Report

Massive thrombosis and phlegmasia cerulea dolens while taking rivaroxaban: case report and review

Trombose maciça e flegmasia cerúlea dolens durante o uso de rivaroxabana: relato de caso e revisão

Diego Chemello; Larissa Rosa; Amanda Faria de Araujo; Pedro Cargnelutti de Araujo; Luiz Carlos Carneiro Pereira; Suélen Feijó Hillesheim; Marco Aurélio Lumertz Saffi

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Abstract

Abstract: Our study describes a fatal case of phlegmasia cerulea dolens and massive venous thrombosis in a patient taking rivaroxaban regularly to treat cerebral venous sinus thrombosis. Blood tests samples were positive for lupus anticoagulant. The unique evolution of the case, as well as the positivity for lupus anticoagulant, raises the possibility of an acquired hypercoagulation syndrome. We highlight the fact that the test recommended as the first line for lupus anticoagulant diagnosis (dilute Russell viper venom time) is the most affected by rivaroxaban, leading to a high prevalence of false-positive results. We also discuss potential diagnoses for the current case and review the current state-of-the-art of use of the novel oral anticoagulation agents in this unusual situation. So far, there are no recommendations to use such agents as first options in cerebral venous sinus thrombosis or in hypercoagulation syndromes.

Keywords

anticoagulants, venous thrombosis, sinus thrombosis, intracranial

Resumo

Resumo: Nosso estudo descreve um caso fatal de flegmasia cerúlea dolens e trombose venosa maciça em um paciente usando regularmente rivaroxabana para o tratamento de trombose de seio venoso cerebral. A investigação laboratorial foi positiva para o anticoagulante lúpico. A evolução única do caso aumenta a possibilidade de uma síndrome de hipercoagulabilidade adquirida, bem como a positividade para o anticoagulante lúpico. Destacamos o fato de que o teste recomendado como primeira linha para o diagnóstico de anticoagulante lúpico (veneno de víbora de Russel diluído) é o mais afetado pela rivaroxabana, levando a uma alta prevalência de resultados falso-positivos. Também discutimos os potenciais diagnósticos para o presente caso e revisamos o estado da arte atual dos novos agentes de anticoagulação oral usados nessa situação incomum. Até o presente momento, não há recomendações para o uso de tais agentes como primeira opção na trombose de seios venosos cerebrais ou nas síndromes de hipercoagulação.
 

Palavras-chave

anticoagulantes, trombose venosa, trombose dos seios intracranianos

References

1 King AB, O’Duffy AE, Kumar AB. Heparin resistance and anticoagulation failure in a challenging case of cerebral venous sinus thrombosis. Neurohospitalist. 2016;6(3):118-21. http://dx.doi.org/10.1177/1941874415591500. PMid:27366296.

2 Ferro JM, Bousser MG, Canhao P, et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis - endorsed by the European Academy of Neurology. Eur J Neurol. 2017;24(10):1203-13. http://dx.doi.org/10.1111/ene.13381. PMid:28833980.

3 Kearon C, Akl EA, Ornelas J, et al. Antithrombotic therapy for VTE disease: CHEST guideline and expert panel report. Chest. 2016;149(2):315-52. http://dx.doi.org/10.1016/j.chest.2015.11.026. PMid:26867832.

4 Steffel J, Verhamme P, Potpara TS, et al. The 2018 European Heart Rhythm Association Practical Guide on the use of non-vitamin K antagonist oral anticoagulants in patients with atrial fibrillation. Eur Heart J. 2018;39(16):1330-93. http://dx.doi.org/10.1093/eurheartj/ehy136. PMid:29562325.

5 Saposnik G, Barinagarrementeria F, Brown RD Jr, et al. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011;42(4):1158-92. http://dx.doi.org/10.1161/STR.0b013e31820a8364. PMid:21293023.

6 Geisbüsch C, Richter D, Herweh C, Ringleb PA, Nagel S. Novel factor xa inhibitor for the treatment of cerebral venous and sinus thrombosis: first experience in 7 patients. Stroke. 2014;45(8):2469-71. http://dx.doi.org/10.1161/STROKEAHA.114.006167. PMid:25070963.

7 Mendonça MD, Barbosa R, Cruz-e-Silva V, Calado S, Viana-Baptista M. Oral direct thrombin inhibitor as an alternative in the management of cerebral venous thrombosis: a series of 15 patients. Int J Stroke. 2015;10(7):1115-8. http://dx.doi.org/10.1111/ijs.12462. PMid:25708372.

8 Rao SK, Ibrahim M, Hanni CM, et al. Apixaban for the treatment of cerebral venous thrombosis: a case series. J Neurol Sci. 2017;381:318-20. http://dx.doi.org/10.1016/j.jns.2017.09.007. PMid:28991706.

9 Ferro JM, Coutinho JM, Dentali F, et al. Safety and efficacy of dabigatran etexilate vs dose-adjusted warfarin in patients with cerebral venous thrombosis: a randomized clinical trial. JAMA Neurol. 2019;76(12):1457. http://dx.doi.org/10.1001/jamaneurol.2019.2764. PMid:31479105.

10 Connolly SJ, Ezekowitz MD, Yusuf S, et al. Dabigatran versus warfarin in patients with atrial fibrillation. N Engl J Med. 2009;361(12):1139-51. http://dx.doi.org/10.1056/NEJMoa0905561. PMid:19717844.

11 Schulman S, Kearon C, Kakkar AK, et al. Dabigatran versus warfarin in the treatment of acute venous thromboembolism. N Engl J Med. 2009;361(24):2342-52. http://dx.doi.org/10.1056/NEJMoa0906598. PMid:19966341.

12 Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306. http://dx.doi.org/10.1111/j.1538-7836.2006.01753.x. PMid:16420554.

13 Martinuzzo ME, Barrera LH, D'Adamo MA, Otaso JC, Gimenez MI, Oyhamburu J. Frequent false-positive results of lupus anticoagulant tests in plasmas of patients receiving the new oral anticoagulants and enoxaparin. Int J Lab Hematol. 2014;36(2):144-50. http://dx.doi.org/10.1111/ijlh.12138. PMid:24034808.

14 Cohen H, Hunt BJ, Efthymiou M, et al. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol. 2016;3(9):e426-36. http://dx.doi.org/10.1016/S2352-3026(16)30079-5. PMid:27570089.

15 Malec K, Góralczyk T, Undas A. The use of direct oral anticoagulants in 56 patients with antiphospholipid syndrome. Thromb Res. 2017;152:93-7. http://dx.doi.org/10.1016/j.thromres.2016.12.009. PMid:27989533.

16 Betancur JF, Bonilla-Abadia F, Hormaza AA, Jaramillo FJ, Canas CA, Tobon GJ. Direct oral anticoagulants in antiphospholipid syndrome: a real life case series. Lupus. 2016;25(6):658-62. http://dx.doi.org/10.1177/0961203315624555. PMid:26743321.

17 Dufrost V, Risse J, Reshetnyak T, et al. Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis. Autoimmun Rev. 2018;17(10):1011-21. http://dx.doi.org/10.1016/j.autrev.2018.04.009. PMid:30103045.

18 Pengo V, Banzato A, Bison E, Zoppellaro G, Padayattil Jose S, Denas G. Efficacy and safety of rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome: rationale and design of the Trial on Rivaroxaban in AntiPhospholipid Syndrome (TRAPS) trial. Lupus. 2016;25(3):301-6. http://dx.doi.org/10.1177/0961203315611495. PMid:26466613.

19 Pengo V, Denas G, Zoppellaro G, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018;132(13):1365-71. http://dx.doi.org/10.1182/blood-2018-04-848333. PMid:30002145.

20 Woller SC, Stevens SM, Kaplan DA, et al. Apixaban for the secondary prevention of thrombosis among patients with antiphospholipid syndrome: study rationale and design (ASTRO-APS). Clin Appl Thromb Hemost. 2016;22(3):239-47. http://dx.doi.org/10.1177/1076029615615960. PMid:26566669.

21 Woller SC, Stevens SM, Kaplan DA, Rondina MT. Protocol modification of apixaban for the secondary prevention of thrombosis among patients with antiphospholipid syndrome study. Clin Appl Thromb Hemost. 2018;24(1):192. http://dx.doi.org/10.1177/1076029617729214. PMid:28893087.

22 Cohen H, Hunt BJ, Efthymiou M, et al. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol. 2016;3(9):e426-36. http://dx.doi.org/10.1016/S2352-3026(16)30079-5. PMid:27570089.

23 Schofield JR, Hassell K. Dosing considerations in the use of the direct oral anticoagulants in the antiphospholipid syndrome. J Clin Pharm Ther. 2018;43(1):104-6. http://dx.doi.org/10.1111/jcpt.12582. PMid:28656623.
 


Submitted date:
04/03/2020

Accepted date:
06/18/2020

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