P1660: PERIPROCEDURAL MANAGEMENT IN COMBINED DEFICIENCY OF FACTOR V AND FACTOR VIII: EXPERIENCE FROM A SINGLE CENTER IN SPAIN

Abstract

Background: Inherited combined factor V (FV) and factor VIII (FVIII) deficiency is a rare disorder with typically mild bleeding symptoms. There is limited data about replacement therapy which is usually only required before surgical procedures or traumatic hemorrhages. Aims: Revision of hemostatic treatment and bleeding control in FV+FVIII deficiency patients after surgical procedures. Evaluation of effectiveness and possible adverse events. Methods: We described 3 patients with combined mild-moderate FV+FVIII deficiency. Characteristics of the patients are described in Table 1. There were 8 relevant surgical procedures between 2014 and 2021 performed with previous administration of frozen fresh plasma and/or FVIII replacement. Antifibrinolytic agents were used depending on the patient comorbidities and surgical site. Characteristics of procedures are described in Table 2. Table 1 - Age at diagnosis (years) Gender FV FVIII Gene(s) involved PATIENT 1 2 Female 8-12% 12-17% Homozigous for LMAN1 mutation PATIENT 2 51 Male 13-15% 29-31% Homozigous for LMAN1 mutation PATIENT 3 Childhood onset Male 14-20% 25-29% Unknown Results: No relevant bleeding events were documented and all procedures were successfully completed. FFP dosage has been adapted not only to the FV level but also to patient comorbidities to avoid congestive cardiac symptoms. Desmopressin has only been used in one patient for a minor procedure. Image:Summary/Conclusion: FFP and FVIII factor replacement is a safe measure for periprocedural preparation, and the correspondent dose can be adapted to patient characteristics including bleeding phenotype, comorbidities and surgical site without increasing hemorrhagic risks. Desmopressin maybe underused.