A retrospective analysis of 157 surgical procedures performed without replacement therapy in 83 unrelated factor VII-deficient patients.

BACKGROUND

Inherited factor (F)VII deficiency is the commonest of the rare bleeding disorders, with a wide set of hemorrhagic features. Other than for the severe clinical forms (for which treatment guidelines are well defined), consistent recommendations regarding perioperative replacement management do not exist for mild and asymptomatic FVII-deficient patients.

OBJECTIVES

The present study aimed to evaluate the influence of bleeding history, FVII procoagulant activity levels (FVII:C) and the type of surgical procedure on the management of inherited FVII-deficient patients before surgery.

PATIENTS: One hundred and fifty-seven surgical procedures, performed without replacement therapy, in 83 unrelated FVII-deficient patients (median FVII:C=5%, range 0.6%-35%) were analyzed.

RESULTS

The overall bleeding rate was 15.3%. We found a significant relationship between previous deep traumatic hematomas and bleeding at surgery, although relationships with previous common epistaxis, easy bruising and menorrhagia were not significant. The receiver-operating characteristic (ROC) curve analysis performed on the first 83 procedures allowed us to define a cut-off value of 7% with a sensitivity of 87% (negative predictive value: 94%). To enhance the sensitivity, and to take into account the potential variation resulting from non-standardized FVII:C measurements, we would suggest applying a threshold of 10%.

CONCLUSION

We have proposed recommendations for the perioperative management of FVII-deficient patients based on FVII:C levels, a thorough bleeding history and the type of surgery involved. By applying these recommendations, minor procedures that risk only external or controlled hemorrhage can be performed in asymptomatic or mildly affected adults, even those with FVII:C levels below 10%.