The risk of this second issue is highlighted by the high frequency of bleeding symptoms reported by the general population [2, 3]. In response to these challenges, a number of attempts have been made to standardize bleeding histories. Over the years, multiple investigators have made attempts to standardize bleeding histories by identifying questions that best distinguish between affected and unaffected individuals. In

1990, Higham and colleagues published the Pictorial Bleeding Assessment Chart (PBAC) which allows women with heavy menstrual bleeding selleckchem to track the number of pads or tampons used for a menstrual period as well as the degree of soiling [4]. Based on that information, a score is generated and PBAC scores ≥100 correlate with menorrhagia, defined as ≥80 mL menstrual blood loss. In 1995, Sramek and colleagues published their experience with a bleeding questionnaire

that was administered to patients known to have a bleeding disorder and to a group of normal controls [5]. The most informative questions, in terms of discrimination, were about bleeding following traumatic events, such as tonsillectomy or dental extraction (but not childbirth), and the presence of a bleeding disorder in a family member. In 2005, the International Society on Thrombosis and Haemostasis (ISTH) Scientific and Standardization Committee (SSC) on von Willebrand factor (VWF) established a set of provisional criteria for the diagnosis of von Willebrand disease (VWD) type 1 including the threshold that must be met for mucocutaneous bleeding symptoms to be considered significant [6]. Over time, the field find more has increasingly focused on quantitative assessments of bleeding, and on the need for standardization. Building on the ISTH provisional MCE公司 criteria, Rodeghiero et al. developed and validated a bleeding assessment tool (BAT) for the diagnosis of type 1 VWD in a primarily adult population [7]. This bleeding questionnaire subsequently underwent a series of modifications, including one by Bowman et al. specifically

aimed at decreasing administration time [8] and culminating with the publication of the ISTH-BAT in 2010 [9]. Studies focused on evaluating the utility of these and other BATs for use in patients with RBDs have begun. As a first step, a classification system for RBDs based on the association between coagulant factor activity and clinical bleeding severity was published by Peyvandi et al. in 2012 [10]. So far, the largest study to date was published by the European Network of Rare Bleeding Disorders (EN-RBD) Group [11]. The objective of this study was to explore the relationship between coagulation factor levels and bleeding severity in patients with RBDs using data from 489 patients registered with the EN-RBD. Clinical bleeding episodes were classified into four categories of severity following consensus. Strong correlations were identified for deficiencies of fibrinogen, FX, FXIII and FV+FVIII.

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