title: Bleeding risk of surgery and its prevention in patients with inherited platelet disorders.
abstract: Excessive bleeding at surgery is a feared complication in patients with inherited platelet disorders. However, very few studies have evaluated the frequency of surgical bleeding in these hemorrhagic disorders. We performed a worldwide, multicentric, retrospective study to assess the bleeding complications of surgery, the preventive and therapeutic approaches adopted, and their efficacy in patients with inherited platelet disorders: the Surgery in Platelet disorders And Therapeutic Approach (SPATA) study. We rated the outcome of 829 surgical procedures carried out in 423 patients with well-defined forms of inherited platelet disorders: 238 inherited platelet function disorders and 185 inherited platelet number disorders. Frequency of surgical bleeding was high in patients with inherited platelet disorders (19.7%), with a significantly higher bleeding incidence in inherited platelet function disorders (24.8%) than in inherited platelet number disorders (13.4%). The frequency of bleeding varied according to the type of inherited platelet disorder, with biallelic Bernard Soulier syndrome having the highest occurrence (44.4%). Frequency of bleeding was predicted by a pre-operative World Health Organization bleeding score of 2 or higher. Some types of surgery were associated with a higher bleeding incidence, like cardiovascular and urological surgery. The use of pre-operative pro-hemostatic treatments was associated with a lower bleeding frequency in patients with inherited platelet function disorders but not in inherited platelet number disorders. Desmopressin, alone or with antifibrinolytic agents, was the preventive treatment associated with the lowest bleedings. Platelet transfusions were used more frequently in patients at higher bleeding risk. Surgical bleeding risk in inherited platelet disorders is substantial, especially in inherited platelet function disorders, and bleeding history, type of disorder, type of surgery and female sex are associated with higher bleeding frequency. Prophylactic pre-operative pro-hemostatic treatments appear to be required and are associated with a lower bleeding incidence.
authors.affiliation: Department of Medicine, Section of Internal and Cardiovascular Medicine, University of Perugia, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Orsini
authors.affiliation: Department of Internal Medicine, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Italy.
authors.forename: Patrizia
authors.initials: P
authors.lastname: Noris
authors.affiliation: Department of Medicine, Section of Internal and Cardiovascular Medicine, University of Perugia, Italy.
authors.forename: Loredana
authors.initials: L
authors.lastname: Bury
authors.affiliation: Hematología Investigación, Instituto de Investigaciones Médicas Alfredo Lanari, Universidad de Buenos Aires, CONICET, Argentina.
authors.forename: Paula G
authors.initials: PG
authors.lastname: Heller
authors.affiliation: La Sapienza University of Rome, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Santoro
authors.affiliation: Haemophilia Centre and Haemostasis Unit, Royal Free Hospital, London, UK.
authors.forename: Rezan A
authors.initials: RA
authors.lastname: Kadir
authors.affiliation: Unidad de Hematología, Hospital Universitario La Paz-IDIPaz, Madrid, Spain.
authors.forename: Nora C
authors.initials: NC
authors.lastname: Butta
authors.affiliation: Department of Medicine, Section of Internal and Cardiovascular Medicine, University of Perugia, Italy.
authors.forename: Emanuela
authors.initials: E
authors.lastname: Falcinelli
authors.affiliation: Department of Medicine, Section of Internal and Cardiovascular Medicine, University of Perugia, Italy.
authors.forename: Ana Rosa
authors.initials: AR
authors.lastname: Cid
authors.affiliation: Clinica Medica 1 - Medicina Interna CLOPD, Dipartimento Assistenziale Integrato di Medicina, Azienda-Ospedale Università di Padova and Dipartimento di Medicina, Università di Padova, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Fabris
authors.affiliation: Consultations d'Hémostase - CRTH, CHU de Nantes, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Fouassier
authors.affiliation: Department of Hematology, Kitasato University School of Medicine, Sagamihara, Japan.
authors.forename: Koji
authors.initials: K
authors.lastname: Miyazaki
authors.affiliation: Servicio de Hematología y Oncología Médica, Hospital Universitario Morales Meseguery Centro Regional de Hemodonación, IMIB-Arrixaca, Universidad de Murcia, Murcia 30003 and Grupo de Investigación CB15/00055 del Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Instituto de Salud Carlos III (ISCIII), Madrid, Spain.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Lozano
authors.affiliation: Department of Hematology-Oncology, School of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Pamela
authors.initials: P
authors.lastname: Zúñiga
authors.affiliation: Service d'Hématologie Biologique Cochin Hospital, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Flaujac
authors.affiliation: Medicina III, ASST Santi Paolo e Carlo, Dipartimento di Scienze della Salute, Università degli Studi di Milano, Italy.
authors.forename: Gian Marco
authors.initials: GM
authors.lastname: Podda
authors.affiliation: Department of Hematology, Hospital San Pedro de Alcántara, Cáceres, Spain.
authors.forename: Nuria
authors.initials: N
authors.lastname: Bermejo
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Armand Trousseau Children's Hospital, French Reference Centre for Inherited Platelet Disorders, Paris, France.
authors.forename: Remi
authors.initials: R
authors.lastname: Favier
authors.affiliation: Hematological Laboratory, Maastricht University Medical Centre, Maastricht, The Netherlands.
authors.forename: Yvonne
authors.initials: Y
authors.lastname: Henskens
authors.affiliation: Department of Biology and Haematology, Centre Hospitalier Universitaire Dijon, France.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: De Maistre
authors.affiliation: Hemostasis and Thrombosis Unit, Institute of Internal Medicine, Policlinico Agostino Gemelli-Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Erica
authors.initials: E
authors.lastname: De Candia
authors.affiliation: School of Clinical Sciences, University of Bristol, UK.
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Mumford
authors.affiliation: Cerrahpasa Medical Faculty, Pediatric Hematology Department, Istanbul, Turkey.
authors.forename: Gul Nihal
authors.initials: GN
authors.lastname: Ozdemir
authors.affiliation: Gülhane Military Medical Faculty, Pediatric Hematology Department, Ankara, Turkey.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Eker
authors.affiliation: Reference Centre for Platelet Disorders, Bordeaux University Hospital Centre, Rythmology and Cardiac Modeling Institute (LIRYC), Xavier Arnozan Hospital, Pessac, France.
authors.forename: Paquita
authors.initials: P
authors.lastname: Nurden
authors.affiliation: Centre Régional de Traitement des Hémophiles, Centre Hospitalier Universitaire de Rennes, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Bayart
authors.affiliation: 1 Division of Hematology, Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PN, USA.
authors.forename: Michele P
authors.initials: MP
authors.lastname: Lambert
authors.affiliation: Department of Pediatrics, Division of Hematology, Weill Cornell Medicine, New York, NY, USA.
authors.forename: James
authors.initials: J
authors.lastname: Bussel
authors.affiliation: Department of Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Germany.
authors.forename: Barbara
authors.initials: B
authors.lastname: Zieger
authors.affiliation: Hematology Department, S. Bortolo Hospital, Vicenza, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Tosetto
authors.affiliation: Department of Internal Medicine, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Melazzini
authors.affiliation: Hematología Investigación, Instituto de Investigaciones Médicas Alfredo Lanari, Universidad de Buenos Aires, CONICET, Argentina.
authors.forename: Ana C
authors.initials: AC
authors.lastname: Glembotsky
authors.affiliation: Department of Internal Medicine, IRCCS Policlinico S. Matteo Foundation, University of Pavia, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Pecci
authors.affiliation: Medicina III, ASST Santi Paolo e Carlo, Dipartimento di Scienze della Salute, Università degli Studi di Milano, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Cattaneo
authors.affiliation: Centre de Référence des Pathologies Plaquettaires (CRPP), Service d'Hématologie Biologique, CHU Robert Debré, AP-HP, Paris, France.
authors.forename: Nicole
authors.initials: N
authors.lastname: Schlegel
authors.affiliation: Department of Medicine, Section of Internal and Cardiovascular Medicine, University of Perugia, Italy paolo.gresele@unipg.it.
authors.forename: Paolo
authors.initials: P
authors.lastname: Gresele
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001791:Blood Platelet Disorders / Q000150:complications* / Q000151:congenital* / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D005260:Female; D006470:Hemorrhage / Q000453:epidemiology / Q000209:etiology* / Q000517:prevention & control*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D063868:Patient Outcome Assessment; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control; D011292:Premedication / Q000379:methods; D018570:Risk Assessment; D012307:Risk Factors; D013514:Surgical Procedures, Operative / Q000009:adverse effects* / Q000379:methods; D016896:Treatment Outcome; D055815:Young Adult