Risk of Bleeding and Inhibitor Development After Circumcision in Minimally Treated Severe Hemophilia A Patients: A One Year Prospective Study,

Abstract

4653 Background Circumcision is a cultural practice for males in the Middle-East during first weeks of life. All parents of hemophilics are eager to do circumcision to their sibs, however, it may carry a risk for development of factor VIII inhibitors as well as risk of excessive bleeding. Objective To evaluate post-circumcision bleeding and assess incidence and time of inhibitor development over 12 months follow-up period of minimally treated severe hemophilia A patients. Patients and methods This prospective analysis has been conducted on eighteen minimally treated patients with severe hemophilia A (age range 8–36 months) with a median age of 18 months, who underwent circumcision during 2009 and twenty four age matched non circumcised patients minimally treated severe hemophilia A. Both groups were followed up for12 months from study entry and all were treated on demand therapy with a single plasma-derived factor VIII product. Hemophilic patients who underwent circumcision were inhibitor negative except two with low- titer inhibitor(3.3 and 4.4 BU/ml) respectively. One hour before the operation, intravenous tranexamic acid (25 mg/ kg) and first dose of factor concentrate (25 unit / Kg) were given to the patients. After reaching a trough plasma factor level more than 90%, patients underwent circumcision using general anesthesia and same surgical technique for all. Bolus injections of factor VIII concentrate were repeated in a dose of (25 units / Kg ) twenty four hours after operation. However, the two patients with inhibitors were given factor VIII concentrate in a dose of (50 units /Kg) with an extra dose at forty-eight hours. Another dose of factor concentrate (25 units/ Kg) was given just before removal of gauze dressing at 5th −7th day post operative. Follow up for inhibitor development was assessed every 8 exposure days (EDs) for 12 months or 100 EDs whichever comes first. Results: Of the eighteen patients enrolled, only one of the 2 patients with low- titer inhibitor had postoperative bleeding at day 5 and 7 respectively. First attack responded to a single dose of factor administration (50 units/Kg), whereas haemostasis was achieved in the second episode after a single dose of Recombinant Factor VIIa (90 microgram/kg) and applying absorbable haemostatic agent (gelatin sponge) and binding. None of the other patients had any bleeding or infection at site of surgery. High -titer inhibitors developed in three patients (16.6 % ) during the follow-up; after 8, 16 and 40 EDs respectively in contrast to four patients (16.6 %) developed high titer inhibitor in the non circumcised group; after a median of 16 exposure days (range 8– 60 EDs). Conclusion: Our study has shown that bleeding following circumcision was absent except in low- titer inhibitor patient necessitating administration of Recombinant Factor VIIa. Moreover, circumcision was not a risk for development of inhibitor where the incidence of high- titer inhibitors during12 months follow up was low in this cohort of minimally treated patients and comparable to non circumcised group. Disclosures: No relevant conflicts of interest to declare.