Bleeding risk of surgery in patients with inherited platelet function disorders (IFPD): outcome of 389 surgeries in 205 patients.

PAOLO GRESELE ; PATRIZIA NORIS; SARA ORSINI; PAULA HELLER; CRISTINA SANTORO; LOREDANA BURY; OMAMOTO OTOMEWO; DIEGO MEZZANO; EMANUELA FALCINELLI; NORA BUTTA; ANA ROSA CID; JOSE RIVERA POZO; KOJI MIYAZAKI; FABRIZIO FABRIS; MARC FOUASSIER; CLAIRE FLAUJACGUL NIHAL OZDEMIR; FEDERICA MELAZZINI; REMI FAVIER; MARCO CATTANEO; NICOLE SCHLEGEL

Congreso; 20th Congress of the European Hematology Association; 2015

BackgroundIPFD are a heterogeneous group of rare mucocutaneous bleeding diseases of variable clinical severity. Excessive bleeding at surgery is a feared complication for bleeding disorders. While studies on the bleeding risk of surgical procedures in other hemorrhagic disorders, like hemophilia or von Willebrand disease, have been conducted, very few and small studies have evaluated the bleeding risk associated with surgery in IPFD.AimsOur aim was to evaluate the bleeding complications of surgical procedures in patients with IPFD, the therapeutic approaches adopted for the prevention/treatment of hemorrhage and their efficacy.MethodsRetrospective, multicentre worldwide study involving clinical centers managing IPFD. Participants were asked to examine retrospectively their patient records and to enroll all cases who had undergone surgery fulfilling strict inclusion criteria. Patients had to have a definite IPDF diagnosis, confirmed according to well-defined laboratory and/or molecular genetic criteria. All types of surgical procedures, including biopsies and dental extractions, were admitted. Bleeding tendency before surgery was scored by the WHO bleeding scale, while the extent of bleeding at surgery was assessed by the BARC classification, subjective evaluation from the surgeon, and/or duration of bleeding at surgery. Information about prophylactic antihemorrhagic preparation and emergency treatment of bleeding was requested.Results205 patients (Age 5 to 86, 56.6% females) from 36 centers with 13 different forms of IPFD were enrolled: 43.4% had Glanzmann Thrombasthenia (GT), 22.4% Primary Secretion Defect (PSD), 6.8% combined α-δ granule deficiency (αδ-SPD), 5.4% Hermansky-Pudlak syndrome (HPS), 4.4% Gray platelet syndrome (GPS). Data from 389 surgeries were collected, the most frequent being dental extraction (26.7%), adenotonsillectomy (3.3%), appendectomy (2.8%), endoscopic polipectomy (2.8%). Major procedures, like cholecystectomy (2.6%), hernia repair (2.6%), gastrectomy (2.3%), hysterectomy (1.8%), were also reported. The frequency of excessive bleeding at surgery was 19.8% by subjective evaluation, 19.7% by the BARC classification and 23.1% by the combination of the two. High frequency of excessive bleeding was observed in GT (29.5% of 183 procedures), HPS (27.3% of 22) and α-δ SPD (12.5% of 32). Frequency of excessive bleeding in some forms remains uncertain due to the low number, but some of them (thromboxane A2 receptor defect, CalDAG, platelet-type von Willebrand Disease) appear to be at high risk. Excessive bleeding at surgery was significantly predicted by presurgical WHO bleeding score=3 (OR 8.1, 95% CI 1.1-61.8) and =4 (OR 28, 95% CI: 2.9-270.5). Prophylactic platelet transfusions were given in 38.5% of procedures and antifibrinolytic agents or DDAVP in 32.9%, and reduced significantly the risk of excessive bleeding (OR 0.3, 95% CI: 0.2-0.5). Emergency treatment was administered in 85.5% of the procedures with excessive bleeding (platelet transfusions in 50.6%, antifibrinolytic agents in 23.4%, FVIIa in 7.8%, DDAVP in 3.9%, other in 14.3%), and was successful in 81.8% of cases.SummaryIPFD are associated with a significant surgical bleeding risk. The risk of excessive bleeding varies according to the diagnosis, with GT having the highest risk, and is predicted by the preoperative WHO bleeding score. Preoperative prohemostatic prophylactic treatments prevent excessive bleeding in most cases. Treatment of excessive bleeding with platelet transfusions and/or other measures stops excessive bleeding in most, but not all, treated cases.