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.