Pulmonary Vascular Disease |

Thrombophilia vs. Hemophilia? How About Both? FREE TO VIEW

Carlos Martinez-Balzano, MD; Emil Tigas, MD; Scott Kopec, MD
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Division of Lung, Allergy and Critical Care Medicine. University of Massachusetts Medical School, Worcester, MA

Chest. 2015;148(4_MeetingAbstracts):970A. doi:10.1378/chest.2241823
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SESSION TITLE: Pulmonary Vascular Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Venous thromboembolism (VTE) in patients with hemophilia B (HB) may be more common than currently thought. There is no consensus addressing thromboprophylaxis for this population. We report a case of a man with HB and an acute pulmonary embolism (PE) after a surgical procedure.

CASE PRESENTATION: A 51-year-old man with history of mild HB, Brugada syndrome, s/p automated implantable cardioverter-defibrillator (AICD) placement, obesity and smoking, was transferred to our institution with the diagnosis of Candida albicans fungemia, acquired from a percutanoeus vascular catheter removed prior to transfer. Due to concerns of an infected AICD wire based on persistently positive blood cultures despite adequate anti-fungal therapy, the AICD was removed. He received peri-operative infusions of recombinant factor IX (rFIX), 6800 IU loading dose, followed by 6400 IU for 4 days, with monitored levels to prevent bleeding. Seven days later, he developed hypoxemic respiratory failure requiring supplemental oxygen. Repeat blood cultures were negative and chest computed tomography with intravenous contrast demonstrated filling defects in the right pulmonary and left lower lobe segmental arteries with corresponding infarction. Echocardiogram did not show valvular vegetations. The diagnosis of acute PE was made. Septic embolism was considered but PE was favored due to the vascular filling defects and negative blood cultures. Anticoagulation was deemed as contraindicated due to underlying HB, and a retrievable inferior vena cava filter was placed.

DISCUSSION: Older case series of patients with primarily severe hemophilia who underwent orthopedic surgeries had shown almost no VTE, suggesting that this complication is rare. However, recent evidence has challenged this notion. Several studies have shown up to a 10% incidence of DVT in patients with HB. No clear guidelines for thromboprophylaxis for these individuals exist but a physician survey showed that clinicians use pharmacologic prophylaxis, along with rFIX infusions to prevent bleeding, in up to 50% of surgical cases.

CONCLUSIONS: To our knowledge, this is the seventh reported case of an acute PE in a patient with HB.1 Although a low-risk procedure was done, the patient had immobilization, obesity and fungemia as risk factors. Research addressing the true incidence of VTE and the role for thromboprophylaxis in the hemophiliac population is needed.

Reference #1: Girolami A, et al. Non-catheter associated venous thrombosis in hemophilia A and B. J Thromb Thrombolysis. 2006;21:279-84.

DISCLOSURE: The following authors have nothing to disclose: Carlos Martinez-Balzano, Emil Tigas, Scott Kopec

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