Visual inspection of the aPTT reaction curve as a reliable screening tool for detecting the presence of FVIII and FIX inhibitors
BACKGROUND Specific shapes and slopes of the aPTT reaction curve are characteristic for patients with congenital or acquired FVIII/FIX deficiency. Questionable aPTT results are flagged by the analyzer and should be visually inspected.AIMS To investigate the...
Usefulness of global haemostasis assays in haemophilia A patients with discrepant bleeding phenotype
BACKGROUND / AIMS Traditionally used laboratory methods, i.e. one-stage or chromogenic assays for FVIII activity determination do not always and accurately reflect bleeding severity in haemophilia A (HA) patients. As global haemostasis assays provide overall...
Comparison of the effect of cationic and anionic polyamidoamine dendrimers on components of coagulation system
Background Polyamidoamine (PAMAM) dendrimers, monodispersed polymers with highly branched three-dimensional globular structure and surface functional groups (-NH2, -OH, or -COOH), are of growing interest in nanomedicine, notably in systemic administrations as drug...
Immune tolerance induction rescue (ITI-R) with human-cl rhFVIII in hemophilia A patients and high-titre inhibitors
Backgroud The appearance of inhibitors is the most serious complication in hemophilia A. The primary objective is their eradication. When the primary ITI fails, another ITI-R is often started. We report three cases of ITI-R with the human cell line-derived recombinant...
Prospective Study Of The Immunological Response To Factor VIII In Severe Hemophilia A Patients During Immune Tolerance Induction Treatment
BACKGROUND AND AIMS The development of an immunogenic response to therapeutic FVIII, with the appearance of neutralizing antibodies ("inhibitors"), is the main adverse event in hemophilia A treatment. The only proven and currently available strategy to eliminate...
Accurate factor FVIII concentrate dosing by ideal body weight in overweight and obese hemophilia A patients
Background Under-and especially overdosing of replacement therapy in hemophilia A patients with varying BMI may be prevented using other morphometric variables than actual body weight to dose factor VIII (FVIII) concentrates. Population pharmacokinetics (PK) enables...
The presence of anti-ADAMTS13 autoantibodies in immune-mediated thrombotic thrombocytopenic purpura patients does not hamper correct determination of ADAMTS13 antigen levels
Introduction and Aim Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening microangiopathic disorder, characterized by severe thrombocytopenia, hemolytic anemia and organ ischemia, which is caused by a functional deficiency in the von Willebrand factor...
Single-sample detection of anti-ADAMTS13 autoantibodies using fiber-optic surface plasmon resonance technology
Introduction and Aim Determination of ADAMTS13 activity, antigen and anti-ADAMTS13 autoantibodies is needed for the diagnosis, prognosis and treatment of the rare disease thrombotic thrombocytopenic purpura (TTP) (Figure 1). ADAMTS13 parameters are, however, not...
Analytical Performance Validation of a new screening assay for the deficiency of ADAMTS-13 Activity
Introduction Background Severe deficiency in ADAMTS-13 activity levels (< 0.1 IU/ml) is a specific marker for thrombotic thrombocytopenic purpura (TTP). TTP is an acute life threatening disorder with the highest mortality rate within the first 24 h of admission. It...
BMI is an important determinant of VWF and FVIII levels and bleeding phenotype in patients with von Willebrand disease
Introduction In the general population, BMI is associated with increased VWF levels. Slightly increased VWF levels may have a protective effect on bleeding in VWD patients. The association between BMI, VWF levels and bleeding is unknown in VWD patients.Objective We...
Feasibility of a new fully automated ADAMTS-13 activity assay
Introduction The aim of these studies was to develop a fully automated ADAMTS13 activity assay using a fluorescence resonance energy transfer (FRET) method. For this purpose a new coagulation analyzer, equipped with an optical Quenching module was co-developed....