Pulmonary embolism in a patient with severe factor VII deficiency after femoral neck fracture despite thromboprophylaxis with low-molecular-weight heparin

K. Holstein, M. Voigtländer, C. Dicke, F. Langer (Hamburg, Germany)

Bleeding disorders, coagulation and fibrinolytic factors
Date: 17.02.2017,
Time: 17:15 - 18:15

Objective: Thromboembolic complications are rare in patients with factor VII deficiency and mostly associated with replacement therapy or other thrombotic risk factors. In addition, it is known that residual factor VII activity does not precisely predict bleeding risk.

Methods: We report the case of a 75-year-old male patient with severe factor VII deficiency and pulmonary embolism.

Results: The patient presented after 4 weeks of conservative treatment of femoral neck fracture. Despite the previously known severe factor VII deficiency (FVII:C, < 1%) he obtained thromboprophylaxis with certoparin 3000 anti-Xa units daily. His previous medical history was uneventful in terms of bleeding complications, but no major surgery had been performed; factor VII deficiency was diagnosed in 2003 due to pathological coagulation tests. On admission, because of dyspnoea, tachycardia and hypotension, bilateral pulmonary embolism (PE) and pneumonia were diagnosed. Anticoagulation was initiated with therapeutic unfractionated Heparin (UFH). Two bleeding episodes (epistaxis and after tracheostoma manipulation) were treated successfully with a single dose of plasmatic factor VII concentrate (Immuseven^TM) at 10-20 IU/kg body weight (BW). Eleven days after diagnosis of PE, surgery with hip joint replacement was performed after a single dose of 20 IU/kg BW factor VII without bleeding complications. Anticoagulation with UFH was soon restarted, and he was discharged to rehabilitation with certoparin 8000 anti-Xa units OD. Six weeks after diagnosis of PE, anticoagulation was reduced to a prophylactic dose due to recurrent epistaxis. Further laboratory work-up showed no severe thrombophilia apart from elevated factor VIII and fibrinogen levels. Unfortunately, determination of the FVII antigen level and testing for a F7 gene mutation could not be performed. Native thrombelastometry showed a procoagulant state. Interestingly, we observed increased thrombin generation compared to commercially available factor VII deficient and normal human plasma.

Conclusion: Although severe factor VII deficiency is perceived as a bleeding disorder, patients are not always protected against thromboembolic complications. Thorough individual risk evaluation considering bleeding history and thrombotic risk factors should thus be performed.