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.

Perioperative thrombocytopenia: evidence, evaluation, and emerging therapies

“…A retrospective analysis of bleeding and transfusion with invasive procedures in a heterogenous group of 423 patients with inherited platelet disorders found excessive bleeding risk in those with Bernard-Soulier syndrome, autosomal variant Glanzmann thrombasthenia, and Hermansky-Pudlak syndrome. 36 Bleeding was twice as common in inherited platelet function disorders (24.8% of procedures) compared with inherited thrombocytopenias (13.4% of procedures). The latter were associated with infrequent surgical bleeding until the platelet count was below 68Â10 9 L À1 .…”

“…The latter were associated with infrequent surgical bleeding until the platelet count was below 68Â10 9 L À1 . 36…”

Platelet function testing at low platelet counts: When can you trust your analysis?

“…that the risk of spontaneous bleeding events in severely thrombocytopenic patients is not directly related to the platelet count, 19,20 and that the risk of surgical or obstetric bleeding is higher in patients with inherited disorders of platelet function than in inherited disorders of platelet numbers. 21,22 Also, in another recent study, it was found that although there was an increased incidence of bleeding events during delivery in women with inherited thrombocytopenia and a platelet count of <50 × 10 9 L −1 , the increased incidence did not seem to be linearly correlated with platelet counts, as the odds ratio (OR) for bleeding events showed a nonsignificant trend towards lower incidences of bleeding in the tertile of platelets with a platelet count of 49-80 × 10 9 L −1 in comparison with patients with a platelet count of > 80 × 10 9 L −1 . 23 These observations indicate that clinical decision making needs to be based on knowledge regarding both quantitative and qualitative defects in primary hemostasis.…”

Update on diagnostic testing for platelet function disorders: What is practical and useful?

“…Platelet function disorders (PFD) are clinically important bleeding disorders that are particularly challenging for clinical laboratories to diagnose. Many PFD are associated with increased bleeding scores and increased risks for bleeding . Commonly, laboratory tests for PFD are performed to investigate unexplained bruising, heavy menstrual bleeding, prolonged and excessive bleeding from cuts, nosebleeds, and dental and surgical procedures .…”