Aronis, S

Auerswald, G

Batorova, A

Di Minno, G

Dolce, A

Giansilly Blaizot,M

Ingerslev, J

Mariani, G

NAPOLITANO, Mariasanta

Ruiz de Saez, A

Schved, JF

SIRAGUSA, Sergio

Tagliaferri, A.

Archivio istituzionale della ricerca - Università di Palermo

Factor VII deficiency: evidence that in minor surgery one-day replacement therapy is sufficientG. Mariani1, A. Dolce2, J.Ingerslev Jorgen3, A.Batorova 4, M. Napolitano1, J.F. Schved5,G. Di Minno6 , G. Auerswald7, M. Giansilly-Blaizot8, S.Aronis9, S. Siragusa10, Ruiz de Saez11, A.Tagliaferri12.1Internal Medicine and Hematology-University of L’Aquila, Italy, 2National Institute of Statistics –Palermo-Italy,3University of Arhus, Denmark,4Reference Haemophilia Center, Bratislava, Slovakia, 5Central Laboratory CHU Montpellier, France, 6Internal Medicine-Universityof Naples Federico II, Italy, 7Departments of Pediatrics, University of Bremen, Germany, 8Central Laboratory CHU Montpellier, France, 9Department of Pediatrics, Athens, Greece, 10Haematology Unit, University of Palermo, Italy, 11Haemophilia Center, Caracas, Venezuela, 12Ospedale Regionale, Parma, ItalyIntroductionSurgery-related bleeding is an issue in congenital bleeding disorders and little is known about “minor surgicalinterventions” (endoscopy with biopsies, arthroscopic surgery, lymph node biopsies, skin or breast biopsies, andcomplicated dental work) as defined by Kitchens (1) .Methods:Thirty-eight such procedures were performed in 34 FVII-deficient patients within the frame of the STER, a prospectivetreatment registry (2), TAB.1. Replacement schedules were the choice of the physician in charge. Baseline FVIIcranged: <1-20%. Reported interventions were: Oral Surgery and multiple dental extractions (n=8), single dentalextractions (n=7), ENT and Head & Neck (n=5), catheter insertions (n=3), endoscopic biopsies (n=7) and mixedinterventions (n=8). Replacement therapy (RT) resulted as follows: rFVIIa employed in 29 pt., pdFVII in 8 and FFP in 1.Antifibrinolytics were used in16 pt. (in oral surgery: 11/16).Results:Overall, RT was given for one day in 27 procedures,employed namely in endoscopic biopsies (7/7), catheterinsertions (3/3) and single dental extractions (7/7), TAB.2. Inthe remaining interventions (n=11, i.e. multiple dentalextractions, tonsillectomies) RT was given for 2-10 days.Total rFVIIa dose ranged: 7.2-400 µg/Kg bw; total pFVIIrange: 9-300 IU/Kg. No bleeding or thrombotic events wererecorded. In one patient, heavily treated with different RT,an inhibitor to FVII (max titre: 28 BU) appeared four weeksafter the procedure.References•Kitchens CS. Surgery and hemostasis. in: Kitchens CS, Alving BM, Kessler CM (eds). Consultative Hemostasis and Thrombosis. 2nd edition, 2007. Saunders: Philadelphia. Chapter 37.•Clinicaltrials.gov identifyier # NCT01269138ConclusionFor most of the uncomplicated minorsurgery procedures, one-day RT issufficient with low to intermediatedoses of rFVIIa (15 - 60 µg/Kg/bw). Incomplicated and elaboratedinterventions longer lasting RTschedules may be needed.Males Femalesn (%)symptomatic (%)n (%)symptomatic (%)All        n=3411 3 23 20(32.3) (27.3) (67.6) (86.9)FVIIc levels (%)≤10 0 9 8(0.0) (0.0) (39.1) (88.9)>1–105 1 9 9(45.5) (20.0) (39.1) (100.0)>10–206 2 5 2(54.5) (33.3) (21.7) (40.0)Minor Surgery ProceduresnReplacement Therapy (RT)RT daysTotal n. ofdosesTotal doseµg/Kg     IU/KgAnti-Fibrino-lyticsAdverseeventsOral Surgery  + Multiple extractions8rFVIIa     7pdFVII    1 1 - 4 2 - 10rFVIIa 19 – 60pdFVII   1.35/8 Inhibitor (1 case)Single extractions7rFVIIa     6pdFVII    11 1 - 2rFVIIa  14 – 110pdFVII    3006/7 NOENT and Head & Neck5rFVIIa     4pdFVII    11 - 10 1 - 16rFVIIa  7.2 - 400pdFVII    1122/5 NOCatheters 3pdFVII    2FFP         11 1 - 4pdFVII 18 – 29FFP     50 ml/KgNO NOEndoscopies 7 rFVIIa     7 1 1 - 7 rFVIIa  10 - 300 2/7 NOMixed 8rFVIIa     5pdFVII    31 - 9 1 - 16rFVIIa   15 - 60pdFVII  60 - 3701/8 NOTAB.1: GENERAL DATA                                                           TAB.2  MINOR SURGCAL PROCEDURES AND TREATMENT SCHEDULES

Factor VII deficiency: evidence that in minor surgery one-day replacement therapy is sufficient

https://iris.unipa.it/retrieve/handle/10447/75841/79273/poster%20ISTH%20minor%20surgery.pdf

Factor VII deficiency: evidence that in minor surgery one-day replacement therapy is sufficient

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Archivio istituzionale della ricerca - Università di Palermo