J Extra Corpor Technol 2025, 57, 174--177 Ó The Author( s), published by EDP Sciences, 2025 https:// doi. org / 10.1051 / ject / 2025016
Available online at: ject. edpsciences. org CASE REPORT
Navigating polycythemia vera and factor X deficiency on cardiopulmonary bypass for coronary artery bypass grafting: a case report
Jenna L. Cornibe( BS, CCP) 1, 2,* and Arthur H. Katz( MD) 1 1 Jupiter Medical Center, 1210 S Old Dixie Hwy, Jupiter, FL 33458, USA 2 Perfusion Services, Epic Specialty Staffing, 2250 McGregor Blvd Suite 3300, Fort Myers, FL 33901, USA
Received 23 January 2025, Accepted 27 April 2025
Abstract – Published literature about patients with polycythemia vera( PV) undergoing cardiac surgery while utilizing cardiopulmonary bypass( CPB) is scarce, even more so when coupled with an additional rare bleeding disorder. These patients require a multidisciplinary approach to achieve optimal clinical management. Several cases have been reported of oxygenator failure or thrombosis within the extracorporeal circuit. We present a successful CPB run on a patient with coexisting PV and Factor X deficiency undergoing coronary artery bypass grafting. Key words: Bleeding disorder, Coagulation, Cardiopulmonary bypass, Cardiac surgery, CABG, Hemodilution. Introduction
There is a lack of published literature concerning the polycythemia vera( PV) patient population regarding cardiac surgery, particularly with the utilization of cardiopulmonary bypass( CPB). Due to both their hypercoagulable tendencies perioperatively as well as bleeding in the post-operative timeframes, these patients present a unique challenge for overall clinical management [ 1, 2 ]. Coupling PV with another existing bleeding disorder, such as Factor X deficiency, perioperative management of cardiac surgery becomes extensively complicated [ 3 ].
PV is diagnostically categorized by the Janus kinase( JAK)-2 mutation being present within a bone marrow biopsy [ 1 ]. These PV patients present with increased red blood cell( RBC) circulation from their rare myeloproliferative disease, causing possible arterial thrombosis from hyperviscosity within the blood [ 1 ]. Other common characteristics of PV include platelet( PLT) and leukocyte activation as well as fibrinolytic dysfunction, which are some of the contributing factors to this disease’ s thrombogenesis [ 4 ]. Further progression of PV can lead to myeloid metaplasia or possibly leukemia [ 1 ]. Kaiser et al. previously reported a case series of PV patients who experienced oxygenator failure during their CPB run [ 5 ]. The first case ran above normal activated clotting times( ACT) and utilized hemodilution [ 5 ]. Both reported cases had either oxygenator failure and / or thrombosis seen within the CPB
* Corresponding author: jlynnmed @ gmail. com circuit tubing [ 5 ]. Lehot et al. also reported a PV case on CPB with above normal ACT times, which still revealed high CPB systemic arterial line pressures requiring oxygenator changeout and thrombotic CPB circuit components [ 6 ]. Furthermore, Ursulet et al. reported a patient with increasing hemoglobin( Hb)/ hematocrit( HCT), which caused increasing oxygenator pressures on extracorporeal membrane oxygenation until autologous harvest and hemodilution were incorporated [ 7 ].
Factor X deficiency is also a rare blood disorder. Factor X, produced by the liver, is a vitamin K-dependent serine protease that is paramount for the formation of thrombin [ 8 ]. This deficiency can either be acquired or congenital and can be found on chromosome 13( 13q34) [ 8 ]. A patient’ s Factor X level of 10--40 % of normal would be deemed reasonable for hemostasis, although target levels of Factor X are not established for surgery or treatment [ 8 ]. Antifibrinolytics such as epsilon-aminocaproic acid or tranexamic acid can aid in minor bleeding cases, however, fresh frozen plasma( FFP) or alternative factor replacement concentrates would be needed for severe bleeding [ 9 ].
We report a patient with coexisting PV and Factor X deficiency receiving coronary artery bypass grafting utilizing CPB.
Case report
A 63-year-old, 178 cm, and 87 kg male presented with coronary artery disease, PV, and Factor X deficiency noted with activity of 73 %( a 1:1 mixing study revealed he should be responsive with FFP). Comorbidities included atrial fibrillation
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