Successful carotid endarterectomy in a patient with factor XII deficiency (Hageman disease): a case report

Authors

DOI:

https://doi.org/10.14739/2310-1210.2025.4.324458

Keywords:

carotid endarterectomy, factor XII deficiency, Hageman disease, carotid stenosis, coagulopathy, case report

Abstract

Aim. To present the first documented case of successful carotid endarterectomy in a patient with severe Factor XII deficiency and analyze the features of perioperative management.

Materials and methods. We report a case of a 70-year-old male with critical left internal carotid artery stenosis (90 %) and incidentally discovered severe Factor XII deficiency (activity 0.1 %, activated partial thromboplastin time (aPTT) 235.0 seconds) during preoperative screening. A comprehensive differential diagnosis of prolonged aPTT was performed, including measurement of intrinsic coagulation factor activities and immunological testing. Classical carotid endarterectomy under local anesthesia was performed using a standard perioperative thromboprophylaxis protocol.

Results. The surgery and postoperative period were uneventful with a blood loss of 150 ml. Standard thromboprophylaxis was administered using unfractionated heparin intraoperatively and low molecular weight heparin postoperatively. At 3-month follow-up, ultrasound confirmed satisfactory reconstruction with normal flow parameters.

Conclusions. Isolated factor XII deficiency is not a contraindication for carotid endarterectomy when proper preoperative diagnosis is established, and standard perioperative protocols are followed. A multidisciplinary approach involving a hematologist is essential for successful management of such patients.

Author Biographies

M. O. Slabyi, Danylo Halytsky Lviv National Medical University

MD, Intern at the Department of Surgery, Plastic Surgery and Endoscopy, Faculty of Postgraduate Education

H. Yu. Orel, Danylo Halytsky Lviv National Medical University

MD, Vascular Surgeon, Assistant at the Department of Surgery No. 2

O. M. Slabyi, Danylo Halytsky Lviv National Medical University

MD, Vascular Surgeon, Assistant at the Department of Surgery No. 2

References

  1. GBD 2019 Stroke Collaborators. Global burden of stroke and risk factors, 1990-2019: a systematic analysis for the Global Burden of Disease Study 2019. Lancet Neurol. 2021;20(10):795-820. doi: https://doi.org/10.1016/s1474-4422(21)00252-0
  2. Li L, Scott CA, Rothwell PM; Oxford Vascular Study. Trends in Stroke Incidence in High-Income Countries in the 21st Century: Population-Based Study and Systematic Review. Stroke. 2020;51(5):1372-80. doi: https://doi.org/10.1161/STROKEAHA.119.028484
  3. Mazzolai L, Teixido-Tura G, Lanzi S, Boc V, Bossone E, Brodmann M, et al. 2024 ESC Guidelines for the management of peripheral arterial and aortic diseases. Eur Heart J. 2024;45(36):3538-700. doi: https://doi.org/10.1093/eurheartj/ehae179
  4. Naylor R, Rantner B, Ancetti S, de Borst GJ, De Carlo M, Halliday A, et al. Editor's Choice - European Society for Vascular Surgery (ESVS) 2023 Clinical Practice Guidelines on the Management of Atherosclerotic Carotid and Vertebral Artery Disease. Eur J Vasc Endovasc Surg. 2023;65(1):7-111. doi: https://doi.org/10.1016/j.ejvs.2022.04.011
  5. Kamal AH, Tefferi A, Pruthi RK. How to interpret and pursue an abnormal prothrombin time, activated partial thromboplastin time, and bleeding time in adults. Mayo Clin Proc. 2007;82(7):864-73. doi: https://doi.org/10.4065/82.7.864
  6. Bauça JM, Ajzner É, Cadamuro J, Hillarp A, Kristoffersen AH, Meijer P; Working Group on the Postanalytical Phase of the European Federation of Clinical Chemistry and Laboratory Medicine (EFLM). An international study on activated partial thromboplastin time prolongation. Part 1: Analytical results. Clin Chim Acta. 2022;535:167-73. doi: https://doi.org/10.1016/j.cca.2022.08.024
  7. Ratnoff OD, Colopy JE. A familial hemorrhagic trait associated with a deficiency of a clot-promoting fraction of plasma. J Clin Invest. 1955;34(4):602-13. doi: https://doi.org/10.1172/jci103109
  8. Konrath S, Mailer RK, Renné T. Mechanism, Functions, and Diagnostic Relevance of FXII Activation by Foreign Surfaces. Hamostaseologie. 2021;41(6):489-501. doi: https://doi.org/10.1055/a-1528-0499
  9. Lämmle B, Wuillemin WA, Huber I, Krauskopf M, Zürcher C, Pflugshaupt R, et al. Thromboembolism and bleeding tendency in congenital factor XII deficiency--a study on 74 subjects from 14 Swiss families. Thromb Haemost. 1991;65(2):117-21. doi: https://doi.org/10.1055/s-0038-1647467
  10. Kalinin DV. Factor XII(a) inhibitors: a review of the patent literature. Expert Opin Ther Pat. 2021;31(12):1155-76. doi: https://doi.org/10.1080/13543776.2021.1945580
  11. Schmaier AH, Stavrou EX. Factor XII - What's important but not commonly thought about. Res Pract Thromb Haemost. 2019;3(4):599-606. doi: https://doi.org/10.1002/rth2.12235
  12. Mailer RK, Rangaswamy C, Konrath S, Emsley J, Renné T. An update on factor XII-driven vascular inflammation. Biochim Biophys Acta Mol Cell Res. 2022;1869(1):119166. doi: https://doi.org/10.1016/j.bbamcr.2021.119166
  13. Wheeler AP, Gailani D. Why factor XII deficiency is not protected from thrombosis. Arterioscler Thromb Vasc Biol. 2016;36(6):1053-5. doi: https://doi.org/10.1080/17474086.2016.1191944
  14. Girolami A, Randi ML, Gavasso S, Lombardi AM, Spiezia F. The occasional venous thromboses seen in patients with severe (homozygous) FXII deficiency are probably due to associated risk factors: a study of prevalence in 21 patients and review of the literature. J Thromb Thrombolysis. 2004;17(2):139-43. doi: https://doi.org/10.1023/b:thro.0000037670.42776.cd
  15. Eikelboom JW, Connolly SJ, Bosch J, Dagenais GR, Hart RG, Shestakovska O, et al. COMPASS Investigators. Rivaroxaban with or without Aspirin in Stable Cardiovascular Disease. N Engl J Med. 2017;377(14):1319-30. doi: https://doi.org/10.1056/nejmoa1709118
  16. Bonaca MP, Bauersachs RM, Anand SS, Debus ES, Nehler MR, Patel MR, et al. Rivaroxaban in Peripheral Artery Disease after Revascularization. N Engl J Med. 2020;382(21):1994-2004. doi: https://doi.org/10.1056/nejmoa2000052

Additional Files

Published

2025-08-31

How to Cite

1.
Slabyi MO, Orel HY, Slabyi OM. Successful carotid endarterectomy in a patient with factor XII deficiency (Hageman disease): a case report. Zaporozhye Medical Journal [Internet]. 2025Aug.31 [cited 2026May16];27(4):344-8. Available from: https://zmj.zsmu.edu.ua/article/view/324458

Issue

Section

Case Reports