4  Management of spontaneous and traumatic bleeds

1 Hemostatic treatment of bleeding episodes in factor VII deficiency

Key data on hemostatic treatment of bleeding episodes in factor VII deficiency has been provided by the STER. The patients prospectively evaluated in the STER had 1-20 % residual FVII:C activity and were all symptomatic. In this group, rFVIIa is clearly beneficial in the treatment of spontaneous and traumatic bleeds [1].

Intracranial hemorrhages (ICH) are among the most feared bleeding complications. The prospective evaluation from the STER group registered 9 CNS bleeds (8 spontaneous and 1 traumatic). Eight patients were treated with rFVIIa (a minimum of 3 doses of 15- 30 µg/kg). Clinically, more than one dose was needed to stop the bleeding and resolve symptoms in all but one case. In general, the outcome was found to be in the spectrum of “partly effective” to “excellent” [1].

Based on the reported effectiveness of single doses of rFVIIa, Mariani and colleagues suggest an intermediate dose of 60 µg/kg for optimal efficacy. Antifibrinolytics are rarely used in reports from the STER but we recommend its use in cerebral hemorrhage as well in less severe bleeds as described in appendix B [1].

2 Recommendations for rFVIIa replacement and tranexamic acid in spontaneous and traumatic bleeds

Spontaneous or traumatic bleeds in FVII deficiency can be hemostatically treated following the same principles as described for patients undergoing surgery.

However, considering the seriousness of ICH, rFVIIa may be considered in all patients with reduced FVII levels below 40%. In addition, an intermediate dose of rFVIIa 60 µg/kg may be considered initially in severe bleeds.

Even though there is a lack in published data, continuous infusion of rFVIIa should also be considered for treatment of severe spontaneous or traumatic bleeds.

Please see Appendix B for suggested treatment regimens in specific spontaneous or traumatic bleeds.

1.

Napolitano M, Dolce A, Garrido RP, Batorova A, Karimi M, Platokouki H, et al. Replacement therapy for bleeding episodes in factor VII deficiency: A prospective evaluation. Thrombosis and Haemostasis 2013;109:238–47. doi:10.1160/th12-07-0476.