Diagnosis of suspected inherited platelet function disorders: results of a worldwide survey

摘要: Summary Background Diagnosis of inherited platelet function disorders (IPFDs) is important for appropriate management and to improve epidemiologic clinical knowledge. However, there remains a lack consensus on the diagnostic approach. Objectives To gain knowledge current practices diagnosis IPFD worldwide. Methods A 67-item questionnaire was distributed ISTH members several national hemostasis thrombosis societies. Results A total 202 laboratories from 37 countries participated in survey. The most frequent criterion define patients with suspected history mucocutaneous bleeding no acquired cause, but heterogeneity identification criteria evident. Only 64.5% respondents performed direct interview. On average, each laboratory studied 72 per year. commonly used equipment were light-transmission aggregometer, Platelet Function Analyzer-100, flow cytometer. Screening tests count, peripheral blood smear, aggregometry, Analyzer-100. Second-step cytometry, molecular genetic analysis, electron microscopy. Methodologies varied widely. In total, ~ 14 000 investigated yearly 60% turned out not have defect. Of remaining 40%, only 8.7% received at level. Conclusions Many worldwide are involved IPFD. A large fraction remain without diagnosis. high variability approaches