Isolated Prolonged Activated Partial Thromboplastin Time and Contact Factor Deficiencies: Case Series and Management Review
DOI:
https://doi.org/10.17140/RPAOJ-1-105Keywords:
Prekallikrein (PK), Factor XII, Activated partial thromboplastin time (aPTT), Contact factors, Fibrinolysis, Anticoagulation, Prolonged clotting time, ThrombosisAbstract
Background: Contact factor deficiencies are rare disorders that can cause grossly prolonged activated partial thromboplastin time (aPTT) and activated clotting time (ACT) while rarely affecting in vivo hemostasis. This in vitro laboratory phenomenon poses a particular challenge in surgical procedures that require anticoagulation monitoring.
Case: Here we report two cases of contact factor deficiencies; a 67-year-old morbidly obese female with factor XII deficiency requiring revascularization of a graft and a 58-year-old female with prekallikrein (PK) deficiency undergoing routine muscle biopsy.
Conclusion: Peri-operative anticoagulation monitoring poses a significant challenge in contact factor deficiency patients. Awareness of the challenges of contact factor deficiencies allows for optimal peri-operative management. Emerging literature supports that contact factors play a role in fibrinolysis. Increased surveillance of thrombotic events as well as avoidance of fibrinolytics may be necessary in these patients.
References
Asmis LM, Sulzer I, Furlan M, Lammle B. Prekallikrein de-ficiency: the characteristic normalization of the severely pro-longed aPTT following increased preincubation time is due to autoactivation of factor XII. Thromb Res. 2002; 105(6): 463- 470. doi: 10.1016/S0049-3848(02)00045-2
Schmaier AH. The elusive physiologic role of factor XII. J Clin Invest. 2008; 118(9): 3006-3009. doi: 10.1172/JCI36617
Renne T, Schmaier AH, Nickel KF, Blomback M, Maas C. In vivo roles of factor XII. Blood. 2012; 120(22): 4296-4303. doi: 10.1182/blood-2012-07-292094
Sonnenfeld H, Rousseau J, Leroy B, Scherpereel P. Con-genital factor XII deficiency: a rare cause of increased activated cephalin time. Ann Fr Anesth Reanim. 1985; 4(4): 378-379. doi: 10.1016/S0750-7658(85)80110-6
Shin DY, Lee HR, Kang HJ, Na, II, Chang YH, Yang SH. Prevalent factor XII deficiency in cancer patients with isolated aPTT prolongation. Blood Research. 2015; 50(2): 114-117. doi: 10.5045/br.2015.50.2.114
Halbmayer WM, Haushofer A, Schon R, Mannhalter C, Strohmer E, Baumgarten K, Fischer M. The prevalence of moderate and severe FXII (Hageman factor) deficiency among the normal population: evaluation of the incidence of FXII de-ficiency among 300 healthy blood donors. Thromb Haemost. 1994; 71(1): 68-72. Web site. http://europepmc.org/abstract/ med/8165648. Accessed.
Halbmayer WM, Haushofer A, Radek J, Schon R, Deutsch M, Fischer M. Prevalence of factor XII (Hageman factor) de-ficiency among 426 patients with coronary heart disease await-ing cardiac surgery. Coronary Artery Disease. 1994; 5(5): 451-454. Web site. http://journals.lww.com/coronary-artery/Ab-stract/1994/05000/Prevalence_of_factor_XII__Hageman_fac-tor_.12.aspx. Accessed.
Halbmayer WM, Mannhalter C, Feichtinger C, Rubi K, Fisch-er M. The prevalence of factor XII deficiency in 103 orally an-ticoagulated outpatients suffering from recurrent venous and/or arterial thromboembolism. Thromb Haemost. 1992; 68(3): 285- 290. Web site. http://europepmc.org/abstract/med/1440493. Ac-cessed.
Sollo DG, Saleem A. Prekallikrein (Fletcher factor) deficien-cy. Ann Clin Lab Sci. 1985; 15(4): 279-285. Web site. http:// www.annclinlabsci.org/content/15/4/279.short. Accessed
Colman RW. Activation of Plasminogen by Human Plasma Kallikrein. Biochem Bioph Res Co. 1969; 35(2): 273-279. doi: 10.1016/0006-291X(69)90278-2
Conaglen PJ, Akowuah E, Theodore S, Atkinson V. Implica-tions for cardiac surgery in patients with factor XII deficiency. Ann Thorac Surg. 2010; 89(2): 625-626. doi: 10.1016/j.athorac-sur.2009.07.042
DeBois W, Liu J, Lee L, et al. Cardiopulmonary bypass in patients with pre-existing coagulopathy. J Extra Corpor Tech-nol. 2005; 37(1): 15-22. Web site. http://www.ncbi.nlm.nih.gov/ pmc/articles/PMC4680798/. Accessed.
Kelsey PR, Bottomley J, Grotte GJ, Maciver JE. Congeni-tal factor XII deficiency: successful open heart surgery and an-ticoagulation. Clin Lab Haematol. 1985; 7(4): 379-381. doi: 10.1111/j.1365-2257.1985.tb00053.x
Cankovic L, Steenwyk BL, McGiffin DC, Nielsen VG. Prac-tical approach to anticoagulation for cardiopulmonary bypass in the patient with congenital prolonged activated partial thrombo-plastin time. Blood Coagul Fibrinolysis. 2008; 19(7): 725-726. doi: 10.1097/MBC.0b013e32830891ab
van Veen JJ, Laidlaw S, Swanevelder J, et al. Contact factor deficiencies and cardiopulmonary bypass surgery: detection of the defect and monitoring of heparin. Eur J Haematol. 2009; 82(3): 208-212. doi: 10.1111/j.1600-0609.2008.01191.x
Wood MK. Congenital factor XII deficiency and cardio-pulmonary bypass. Ann Thorac Surg. 1994; 58(5): 1565. doi: 10.1016/0003-4975(94)91976-3
Gerhardt MA, Greenberg CS, Slaughter TF, Stafford Smith M. Factor XII deficiency and cardiopulmonary bypass: use of a novel modification of the activated clotting time to monitor anticoagulation. Anesthesiology. 1997; 87(4): 990-992. Web site. http://anesthesiology.pubs.asahq.org/article.aspx?articleid= 1948727. Accessed.
Downloads
Published
How to Cite
Issue
Section
License

This work is licensed under a Creative Commons Attribution 4.0 International License.
All content published in Research and Practice in Anesthesiology (RPA) is licensed under the Creative Commons Attribution 4.0 International License (CC BY 4.0). This license allows anyone to copy, distribute, transmit, and adapt the work — including for commercial purposes — provided appropriate credit is given to the original author(s) and source.