Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry

Sergio Siragusa, Mariasanta Napolitano, Massimo Morfini, Uri Martinowitz, Jorgen Ingerslev, Francesco Bernardi, Benny Sørensen, Amy Shapiro, Sergio Siragusa, Matteo N. D. Di Minno, Muriel Giansilly-Balizot, Guglielmo Mariani, Alberto Dolce, Angelica Batorova, Guenter Auerswald, Giovanni Di Minno, Mirko Pinotti, Jean Francois Schved, Guglielmo Mariani, Amy Shapiro

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10 Citazioni (Scopus)

Abstract

Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII-deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with <3% FVII:C received a higher number of RT doses (8 vs. 2, P = 0·003) for a longer RT duration (3 days vs. 1 day, P = 0·001), with no difference in RT dose. Similarly, patients with a history of major bleeds received a higher number of RT doses (8·5 vs. 2-3, P = 0·013) for a longer RT duration (2 days vs. 1 day, P = 0·005) as compared to those with a history of minor bleeds or to asymptomatic patients. No difference in RT was found among major and minor surgical procedures. Overall, multivariate analysis showed that history of major bleeding was the only independent predictor of number of RT doses (β = 0·352, P = 0·001) and RT duration (β = 0·405, P = 0·018). Overall, a ≈20 μg/kg perioperative RT was efficacious in 95·5% of cases. The infusion should be repeated ≈8 times in high-risk subsets (i.e. patients with a history of major bleeding).
Lingua originaleEnglish
Numero di pagine8
RivistaBritish Journal of Haematology
Stato di pubblicazionePublished - 2017

All Science Journal Classification (ASJC) codes

  • Hematology

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    Siragusa, S., Napolitano, M., Morfini, M., Martinowitz, U., Ingerslev, J., Bernardi, F., Sørensen, B., Shapiro, A., Siragusa, S., Di Minno, M. N. D., Giansilly-Balizot, M., Mariani, G., Dolce, A., Batorova, A., Auerswald, G., Di Minno, G., Pinotti, M., Schved, J. F., Mariani, G., & Shapiro, A. (2017). Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry. British Journal of Haematology.