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Congenital FVII deficiency and thrombotic events after replacement therapy

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Abstract

Thrombosis has been occasionally described in congenital FVII deficiency. This report deals with patients with FVII deficiency who presented thrombotic events after substitution therapy. At least 12 patients are reported in the literature. In all but two cases thrombosis occurred after prothrombin complex concentrates or plasma derived FVII concentrates. In two instances pulmonary embolism occurred after the administration of large amounts of whole blood. Concomitant prothrombotic risk factors were present in most of these cases (surgery, immobilization, old age, etc.). Personal files allowed us to add another patient who developed bilateral pulmonary embolism after two vials of an aFVII concentrate. In this case also, concomitant risk factors were present, namely surgery for hysterectomy, immobilization. The pulmonary embolism occurred in spite of the congenital FVII deficiency indicating that no sure antithrombotic protection is assured by this defect. The actual needs of substitution therapy in patients with some variants of FVII deficiency is discussed, together with comments on the therapeutic management of the thrombotic events in these patients.

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Acknowledgment

This study was supported in part by the “Associazione Emofilia ed altre Coagulopatie delle Tre Venezie”.

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Authors and Affiliations

  1. Department of Medical and Surgical Sciences, University of Padua Medical School, via Ospedale 105, 35128, Padua, Italy

    Antonio Girolami, Irene Bertozzi & Silvia Vettore

  2. Obstetrics and Gynecology Unit, Belluno City Hospital, Belluno, Italy

    Ignazio Rigoni

  3. Pulmonary Diseases Unit, Belluno City Hospital, Belluno, Italy

    Rodolfo Muzzolon

Authors
  1. Antonio Girolami
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  2. Irene Bertozzi
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  3. Ignazio Rigoni
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  4. Rodolfo Muzzolon
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  5. Silvia Vettore
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Correspondence to Antonio Girolami.

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Girolami, A., Bertozzi, I., Rigoni, I. et al. Congenital FVII deficiency and thrombotic events after replacement therapy. J Thromb Thrombolysis 32, 362–367 (2011). https://doi.org/10.1007/s11239-011-0603-8

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  • DOI: https://doi.org/10.1007/s11239-011-0603-8

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