Blood Res.  2013 Dec;48(4):282-286. 10.5045/br.2013.48.4.282.

Sequential therapy with activated prothrombin complex concentrates and recombinant activated factor VII to treat unresponsive bleeding in patients with hemophilia and inhibitors: a single center experience

• Han MH
• Park YS

• KMID: 2172876
• DOI: http://doi.org/10.5045/br.2013.48.4.282

Abstract

BACKGROUND
Currently, the greatest challenge in hemophilia treatment is managing hemophilia patients with inhibitors. The two main bypassing agents that are used to treat hemophilia patients with inhibitors are activated prothrombin complex concentrates (APCC) and recombinant factor VIIa (rFVIIa). Hemophilia patients with inhibitors can develop bleeding episodes, that are refractory to monotherapy with either APCC or rFVIIa and thus are often difficult to manage.
METHODS
This report describes a retrospective chart review of four hospitalized patients with severe hemophilia and inhibitors who were treated with sequential therapy of APCC and rFVIIa for refractory bleeding. Sequential therapy was defined as the administration of both rFVIIa and APCC within 12 h.
RESULTS
In 5 episodes experienced by 4 patients with inhibitors, bleeding was not controlled by single bypass treatment, but it was controlled when two agents were sequentially administered. Sequential therapy was administered by alternating one APCC dose to 1 to 2 rFVIIa doses, with dosing intervals ranging from 3 to 6 h. All bleeding episodes were controlled within 12 to 24 h. Sequential therapy was discontinued after 2 to 5 days. No adverse clinical events, such as thrombosis, were observed.
CONCLUSION
Sequential therapy with APCC and rFVIIa was efficacious without adverse events; however, attention on thrombosis is needed. In addition, a prospective clinical trial is needed to provide further evidence for this treatment.

Keyword

Hemophilia; Inhibitor

MeSH Terms

Factor VIIa*
Hemophilia A*
Hemorrhage*
Humans
Prothrombin*
Retrospective Studies
Thrombosis
Factor VIIa
Prothrombin

Figure

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  Fig. 1 (A) Chest radiograph that shows a right hemothorax and an enlarged cardiac silhouette; (B) Chest CT that shows a hemopneumothorax with active bleeding; (C) Chest x-ray that shows a residual hemothorax after sequential therapy.

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