TY - JOUR
T1 - Bleeding risk of surgery and its prevention in patients with inherited platelet disorders
AU - Orsini, Sara
AU - Noris, Patrizia
AU - Bury, Loredana
AU - Heller, Paula G.
AU - Santoro, Cristina
AU - Kadir, Rezan A.
AU - Butta, Nora C.
AU - Falcinelli, Emanuela
AU - Cid, Ana Rosa
AU - Fabris, Fabrizio
AU - Fouassier, Marc
AU - Miyazaki, Koji
AU - Luisa Lozano, Maria
AU - Zuniga, Pamela
AU - Flaujac, Claire
AU - Podda, Gian Marco
AU - Bermejo, Nuria
AU - Favier, Remi
AU - Henskens, Yvonne
AU - De Maistre, Emmanuel
AU - De Candia, Erica
AU - Mumford, Andrew D.
AU - Ozdemir, Gul Nihal
AU - Eker, Ibrahim
AU - Nurden, Paquita
AU - Bayart, Sophie
AU - Lambert, Michele P.
AU - Bussel, James
AU - Zieger, Barbara
AU - Tosetto, Alberto
AU - Melazzini, Federica
AU - Glembotsky, Ana C.
AU - Pecci, Alessandro
AU - Cattaneo, Marco
AU - Schlegel, Nicole
AU - Gresele, Paolo
AU - EHA-SWG Thrombocytopenias Platelet
PY - 2017/7
Y1 - 2017/7
N2 - 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.
AB - 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.
KW - BERNARD-SOULIER-SYNDROME
KW - HERMANSKY-PUDLAK-SYNDROME
KW - VON-WILLEBRAND-DISEASE
KW - PERIOPERATIVE MANAGEMENT
KW - GLANZMANN THROMBASTHENIA
KW - DIFFERENT FORMS
KW - FACTOR-VII
KW - THROMBOCYTOPENIA
KW - ELTROMBOPAG
KW - DIAGNOSIS
U2 - 10.3324/haematol.2016.160754
DO - 10.3324/haematol.2016.160754
M3 - Article
C2 - 28385783
SN - 0390-6078
VL - 102
SP - 1192
EP - 1203
JO - Haematologica-the Hematology Journal
JF - Haematologica-the Hematology Journal
IS - 7
ER -