Sports participation and physical activity in patients with von Willebrand disease.

Introduction: Patients with bleeding disorders may experience limitations in sports participation and physical activity. Several studies on sports participation have been performed in haemophilia patients, but studies in patients with von Willebrand disease (VWD) are lacking.

Aim: We assessed the sports participation and physical activity of a large cohort of VWD patients.

Clinically relevant differences between assays for von Willebrand factor activity.

Essentials It is unclear whether there are differences between von Willebrand factor (VWF) activity assays. We compared the four most used VWF activity assays in 661 von Willebrand disease (VWD) patients. All assays correlated excellently, but a discrepant classification was seen in 20% of patients.

Good Feasibility of the New German Blood Donor Questionnaire.

Background: We assessed the effect of the uniform donor questionnaire (UDQ) on deferral rates in first-time and repeat donors. We focused on the introduced question about unprotected sexual contact with a new partner. Another goal was a stratified comparison of the deferral rates of the donor questionnaire (DQ) and UDQ.

Long-term impact of joint bleeds in von Willebrand disease: a nested case-control study.

Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life.

Plasma levels of plasminogen activator inhibitor-1 and bleeding phenotype in patients with von Willebrand disease.

Introduction: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients.

A Simple and Rapid Method for Quality Control of Major Histocompatibility Complex-Peptide Monomers by Flow Cytometry.

Major histocompatibility complex (MHC) multimers are essential tools in T cell immunomonitoring, which are employed both in basic and clinical research, as well as for assessing clinical samples during therapy. The generation of MHC monomers loaded with synthetic peptides is an elaborate and time-consuming process. It would be beneficial to assess the quality of these monomers prior to downstream applications.

Antenatal management in fetal and neonatal alloimmune thrombocytopenia: a systematic review.

Several strategies can be used to manage fetal or neonatal alloimmune thrombocytopenia (FNAIT) in subsequent pregnancies. Serial fetal blood sampling (FBS) and intrauterine platelet transfusions (IUPT), as well as weekly maternal IV immunoglobulin infusion (IVIG), with or without additional corticosteroid therapy, are common options, but optimal management has not been determined. The aim of this systematic review was to assess antenatal treatment strategies for FNAIT.

Clinical and laboratory tests for the diagnosis of heparin-induced thrombocytopenia.

A rapid diagnostic work-up is required in patients with suspected heparin-induced thrombocytopenia (HIT). However, diagnosis of HIT is challenging due to a number of practical issues and methodological limitations. Many laboratory tests and a few clinical scoring systems are available but the individual characteristics and the diagnostic accuracy of these are hard to appraise.

Platelet activation in the presence of neutral protamine Hagedorn insulin: a new feature of antibodies against protamine/heparin complexes.

Unlabelled: Essentials Protamine (PRT) is used to stabilize insulin in neutral protamine Hagedorn (NPH) insulin. The interaction between NPH-insulin, anti-PRT/heparin antibodies and platelets was investigated. Anti-PRT/heparin antibodies activate platelets in presence of NPH-insulin dependent on heparin.

Validation of Immunomonitoring Methods for Application in Clinical Studies: The HLA-Peptide Multimer Staining Assay.

Background: Validated assays are essential to generate data with defined specificity, consistency, and reliability. Although the process of validation is required for applying immunoassays in the context of clinical studies, reports on systematic validation of in vitro T cell assays are scarce so far. We recently validated our HLA-peptide multimer staining assay in a systematic manner so as to qualify the method for monitoring antigen-specific T cell responses after immunotherapy.

Joint surgery in von Willebrand disease: a multicentre cross-sectional study.

Background: Joint bleeds are reported by 23% of von Willebrand disease (VWD) patients and associated with orthopaedic surgery. Limited data are available on joint surgery in VWD.

Aim: To assess the prevalence, indications, management and complications of joint surgery in VWD patients.

Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric-specific bleeding.

The bleeding phenotype of children with von Willebrand disease (VWD) needs to be characterized in detail to facilitate diagnosis during childhood and aid in the planning and assessment of treatment strategies. The objective was to evaluate the occurrence, type, and severity of bleeding in a large cohort of children with moderate and severe VWD. We included 113 children (aged 0-16 years) with Type 1 (n = 60), 2 (n = 44), and 3 (n = 9) VWD with von Willebrand factor (VWF) antigen and/or VWF ristocetin cofactor levels ≤ 30 U/dL from a nation-wide cross-sectional study ("Willebrand in the Netherlands" study).

Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross-sectional study.

Background: Joint bleeds (JB) are reported in a minority of patients with von Willebrand disease (VWD) but may lead to structural joint damage. Prevalence, severity and impact of JB in VWD are largely unknown.

Objectives: The aim of this study was to assess JB prevalence, onset, treatment and impact on health-related quality of life (HR-QoL) and joint integrity in moderate and severe VWD.

CLEC4M and STXBP5 gene variations contribute to von Willebrand factor level variation in von Willebrand disease.

Background: von Willebrand factor (VWF) levels in healthy individuals are influenced by variations in genetic loci other than the VWF gene, whose contribution to VWF levels in patients with von Willebrand disease (VWD) is largely unknown.

Objectives: To investigate the association between single-nucleotide polymorphisms (SNPs), VWF levels, and bleeding phenotype.

Patients/methods: In 364 type 1 VWD and 240 type 2 VWD patients from the nationwide cross-sectional 'Willebrand in The Netherlands' (WiN) study, we studied the association between eight SNPs in STXBP5, SCARA5, ABO, VWF, STAB2, STX2, TC2N, and CLEC4M, and VWF antigen (VWF:Ag), VWF activity (VWF:Act), and bleeding phenotype as assessed with the Tosetto bleeding score.

[Influence of "dosage effect" on unexpected antibody identification].

Objective: This study was to investigate the influence of "dosage effect" on unexpected antibody identification and explore its condition, scope and regularity.

Methods: A total of 40 blood recipient samples containing definite unexpected antibodies were selected by column agglutination technology, then AB fresh plasma was used to dilute the samples to obtain different concentrate liquid. After selecting panel cells which show positive with corresponding unexpected antibody in the serum, "single dosage" antigens were distinguished from "double dosage" ones, and then the antigen-antibody reactions were observed between "single dosage" panel cells and respective diluted recipient samples (by column agglutination technology).

von Willebrand factor propeptide and the phenotypic classification of von Willebrand disease.

Unlabelled: The ratios between von Willebrand factor propeptide (VWFpp) or factor VIII activity (

Fviii: C) and VWF antigen (VWF:Ag) reflect synthesis, secretion, and clearance of VWF. We aimed to define the pathophysiology of 658 patients with type 1, 2, or 3 von Willebrand disease (VWD) with VWF levels ≤30 U/dL from the Willebrand in The Netherlands (WiN) study using the VWFpp/VWF:Ag and

Fviii: C/VWF:Ag ratios. We evaluated the use of VWFpp in the classification and diagnosis of VWD.

Effects of hydroformylation treatment on the storage time and blood group antigen expressions of reagent red blood cells.

Objective: To evaluate the effects of hydroformylation treatment on the storage time and blood group antigen expressions of reagent red blood cells (RBCs).

Material And Methods: RBCs from healthy donors were treated by using various final concentrations of paraformaldehyde (0.01%, 0.

[Serological characteristics and transfusion efficacy evaluation in 61 cases of autoimmune hemolytic anemia].

This study was aimed to analyze the serological characteristics, efficacy and safety of incompatible RBC transfusion in patients with autoimmune hemolytic anemia (AIHA). The patients with idiopathic or secondary AIHA were analyzed retrospectively, then the serological characteristics and the incidence of adverse transfusion reactions were investigated, and the efficacy and safety of incompatible RBC transfusion were evaluated according to the different autoantibody type and infused different RBC components. The results showed that out of 61 cases of AIHA, 21 cases were idiopathic, and 40 cases were secondary.

Study on preparation of universal plasma in Chinese Han population.

Background: Universal plasma proves its importance in emergency situations where it simplifies the logistics by eliminating the need for ABO-match. There are now two available products of ABO-universal plasma internationally. Such product is not available in China yet.

HLA antibody specification using single-antigen beads--a technical solution for the prozone effect.

Background: Substantial progress in human leukocyte antigen antibody specification has been made by the introduction of Luminex single-antigen bead (SAB) assays. This progress was impaired when it turned out that this method is prone to a prozone effect leading to false-negative results in the case of high antibody titers. Testing serum and ethylenediaminetetraacetic acid (EDTA) plasma of one patient in parallel, we observed the prozone effect with the serum sample only.