Search

Introduction: Cerebral microbleeds (CMBs) represent clinically silent haemorrhagic events. Cerebral microbleeds (CMBs) portend negative neurovascular and cognitive outcomes in the general population and are associated with cognitive impairment in persons with haemophilia (PWH). Prevalence, patterns, and risk factors for CMBs in PWH have not been directly compared to persons without coagulopathy. Aim: To examine prevalence, patterns, and risk factors for CMBs in PWH vs normal controls. Methods: Adults with haemophilia A or B and haemostatically normal controls were recruited. Subjects were excluded if taking an antithrombotic agent other than low‐dose aspirin (<100 mg). All subjects underwent T2*MRI of the brain; scans were reviewed independently by two neuroradiologists blinded to subject group to determine the presence of CMBs. Results: We recruited 31 PWH and 32 controls. Human immunodeficiency virus (HIV) and history of hepatitis C virus (HCV) infection were more prevalent in PWH; smoking was more common among controls. Cardiovascular (CV) risk factors were similar between groups. Prevalence of CMBs was 35% in PWH and 25% in controls (P = .42). Among PWH, advanced age, history of HCV infection, and CV risk factors were associated with CMBs. Multiple and large (>5 mm) CMBs were seen only in PWH. Conclusions: Cerebral microbleeds (CMBs) are common in adults with haemophilia, but not clearly more prevalent than in haemostatically normal controls. In PWH, older age, HCV infection, CV risk factors, and the presence of an inhibitor were associated with CMBs. Large CMBs and multiple CMBs may be more prevalent in PWH than in the general population. The clinical impact of CMBs in PWH requires further study. [ABSTRACT FROM AUTHOR]

Introduction Factor VIII (FVIII) or factor IX ( FIX)-deficient haemophilic patients display deficits in platelet and fibrin deposition under flow detectable in microfluidics. Compared to fibrin generation, decreased platelet deposition in haemophilic blood flow is more easily rescued with recombinant factor VIIa ( rFVIIa), whereas rFVIIa requires FXIIa participation to generate fibrin when tissue factor ( TF) is absent. Aims Perfusion of haemophilic whole blood (WB) over collagen/ TF surfaces was used to determine whether rFVIIa/ TF was sufficient to bypass poor FIXa/ FVIIIa function in blood from patients with haemophilia A and B. Methods Whole blood treated with high-dose corn trypsin inhibitor (40 μg mL−1) from seven healthy donors and 10 patients was perfused over fibrillar collagen presenting low or high TF ( TFlow or TFhigh) at wall shear rate of 100 s−1. Results With WB from healthy controls, platelet deposition and fibrin accumulation increased as TF increased. Factor-deficient WB (1-3% of normal) displayed striking deficits in platelet deposition and fibrin formation at either TFlow or TFhigh. In contrast, mildly factor-deficient WB (14-32%) supported fibrin formation under flow on TFhigh/collagen. With either TFlow or TFhigh, exogenously added rFVIIa (20 n m) increased platelet deposition and fibrin accumulation in WB from factor-deficient patients (1-3% of normal) to levels commensurate with untreated healthy WB. Conclusion The absence of FIXa/ FVIIIa in patients with severe haemophilia results in deficits in fibrin formation that cannot be rescued by wall-derived TF ex vivo. The effects of rFVIIa on platelet adhesion and rFVIIa/ TF can act together to reinforce thrombin generation, platelet deposition and fibrin formation under flow. [ABSTRACT FROM AUTHOR]

Introduction von Willebrand disease ( VWD) is one of the most common inherited bleeding disorders. Aim Investigate the impact of the VWD bleeding tendency on in-hospital management of acute coronary syndromes ( ACS). Methods Using discharge data from the National Inpatient Sample ( NIS), the features of presentation and in-hospital treatment among ACS hospital discharges with and without a VWD diagnosis were investigated. A total of 264 case discharges and 705 860 control discharges were identified. Results and Conclusions There was a significantly higher percentage of women among the case discharges compared to the control discharges (59.5% and 39.4%, respectively; P < 0.001). The rate of medical therapy alone [i.e. avoidance of percutaneous coronary intervention ( PCI) or coronary artery bypass grafting ( CABG)] was significantly higher among unstable angina cases than controls (55.0% vs. 46.4%; P = 0.01), and among cases undergoing PCI, bare-metal stents ( BMS) were utilized in preference to drug-eluting stents ( DES) (adjusted OR = 3.5); P < 0.001). No difference in in-hospital death was identified, but reported bleeding among discharges that underwent CABG was higher in cases compared to controls (12.9% vs. 5.2%; P = 0.047). Although medical and interventional management of ACS appears to be well tolerated in the majority of hospitalized patients with VWD, the gender ratio is reversed, interventions and DES are utilized less frequently and procedure-related bleeding may be increased, calling for further study. [ABSTRACT FROM AUTHOR]

Introduction There has been increasing recognition in recent years that female carriers of haemophilia manifest abnormal bleeding; however, data on the use of bleeding assessment tools in this population are lacking. Aim Our objective was to validate the ISTH- BAT in haemophilia carriers to describe bleeding symptoms and allow for comparisons with factor levels and other patient groups. Methods This was a prospective, observational, cross-sectional study performed by members of Global Emerging HEmostasis Panel ( GEHEP). Unselected consecutive haemophilia carriers were recruited and a CRF and the ISTH- BAT were completed by study personnel. Results A total of 168 haemophilia carriers were enrolled: 155 haemophilia A and 13 haemophilia B. The mean age was 40 years (range: 20-82). Carriers had higher mean bleeding scores ( BS) compared with age-matched controls ( n = 46; 5.7 vs. 1.43; P < 0.0001) and Type 3 VWD OC ( n = 32; 3.0; P = 0.009), but lower BS compared with women with Type 1 VWD ( n = 83; 8.7; P < 0.0001). Fifteen carriers reported haemarthrosis, and of those six had normal FVIII/ FIX levels. There was a significant but weak negative correlation between BS and factor level (Spearman's r2 = −0.36, P < 0.001). Conclusion Our results show that haemophilia carriers experience abnormal bleeding, including haemarthrosis. Overall, BS in women with Type 1 VWD > haemophilia carriers > Type 3 VWD OC > controls. Understanding the performance of the ISTH- BAT in this population is a critical step in future research aimed at investigating the underlying pathophysiology of abnormal bleeding, with the ultimate goal of optimizing treatment. [ABSTRACT FROM AUTHOR]