Bleeding risk of surgery and its prevention in patients with inherited platelet disorders. The Surgery in Platelet disorders And Therapeutic Approach (SPATA) study

Excessive bleeding at surgery is a feared complication in patients with inherited platelet disorders (IPD), however very few studies have evaluated the frequency of surgical bleeding in these hemorrhagic disorders. We performed a multicentric, retrospective worldwide study to assess the bleeding complications of surgery, the preventive and therapeutic approaches adopted and their efficacy in patients with IPDs by rating the outcome of 829 surgical procedures carried out in 423 patients with well defined forms of IPD (238 inherited platelet function disorders -IPFD- and 185 inherited platelet number disorders-IPND-). Frequency of surgical bleeding was high in patients with IPD (19.7%), with a significantly higher bleeding incidence in IPFDs (24.8%) than in IPNDs (13.4%). The frequency of bleeding varied according to the type of IPD, with biallelic Bernard Soulier syndrome having the highest occurrence (44.4%), and was predicted by a preoperative WHO bleeding score >/-2. Some types of surgery were associated with a higher bleeding incidence, like cardiovascular and urologic surgery. The use of preoperative prohemostatic treatments was associated with a lower bleeding frequency in patients with IPFD but not in IPNDs. Desmopressin, alone or with antifibrinolytic agents, was the preventive treatment associated with lowest bleedings. Platelet transfusions were used more frequently in patients at higher bleeding risk. Surgical bleeding risk in IPD is substantial, especially in IPFDs, and bleeding history, type of disorder, type of surgery and female gender are associated with higher bleeding frequency. Prophylactic preoperative prohemostatic treatments appear to be required and associated with a lower bleeding incidence. Fil: Orsini, Sara. Università di Perugia; Italia Fil: Noris, Patrizia. University Of Pavia. Policlinico San Matteo; Italia Fil: Bury, Loredana. Università di Perugia; Italia Fil: Heller, Paula Graciela. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Santoro, Cristina. Università degli studi di Roma ; Italia Fil: Kadir RA. Royal Free Hospital; Reino Unido Fil: Butta, Nora C.. Hospital Universitario la Paz; España Fil: Falcinelli, Emanuela. Università di Perugia; Italia Fil: Cid, Ana Rosa. Hospital Universitario y Politecnico ; España Fil: Fabris, Fabrizio. Università di Padova; Italia Fil: Fouassier, Marc. Centre Hospitalier Universitaire de Nantes; Francia Fil: Miyazaki, Koji. Kitasato University. School Of Medicine; Japón Fil: Lozano, Maria Luisa. Universidad Carlos III de Madrid. Instituto de Salud; España Fil: Zuñiga, Pamela. Pontificia Universidad Católica de Chile; Chile Fil: Flaujac, Claire. Cochin Hospital; Francia Fil: Podda, Gian Marco. Università degli Studi di Milano; Italia Fil: Bermejo, Nuria. Hospital ; España Fil: Favier, Remi. French Reference Centre For Inherited Platelet Disorder; Francia Fil: Henskens, Yvonne. Maastricht University. Medical Centre; Países Bajos Fil: De Maistre, Emmanuel. Centre Hospitalier Universitaire Dijon; Francia Fil: De Candia, Erica. Universitá Cattolica Sacro. Policlinico Agostino Gemelli; Italia Fil: Mumford, Andrew. University Of Bristol. School Of Clinical Sciences; Reino Unido Fil: Ozdemir, Gul Nihal. Cerrahpasa Medical Faculty. Pediatric Hematology Depart; Turquía Fil: Eker, Ibrahim. Gülhane Military Medical Faculty. Pediatric Hematology; Turquía Fil: Nurden, Paquita. Xavier Arnozan Hospital; Francia Fil: Bayart, Sophie. Centre Régional de Traitement Des Hémophiles; Francia Fil: Lambert, Michele P. University of Pennsylvania; Estados Unidos Fil: Bussel, James. Weill Cornell Medicine; Estados Unidos Fil: Zieger, Barbara. University Medical Center Freiburg; Alemania Fil: Tosseto, Alberto. San Bortolo Hospital; Italia Fil: Melazzini, Federica. Università degli studi di Pavia. Policlinico San Matteo; Italia Fil: Glembotsky, Ana Claudia. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto de Investigaciones Médicas. Universidad de Buenos Aires. Facultad de Medicina. Instituto de Investigaciones Médicas; Argentina Fil: Pecci, Alessandro. Università degli studi di Pavia. Policlinico San Matteo; Italia Fil: Cattaneo, Marco. Università degli Studi di Milano; Italia Fil: Schlegel, Nicole. Centre Hospitalier Universitaire ; Francia Fil: Gresele, Paolo. Università di Perugia; Italia