Abstract:Accurate diagnosis and classification of von Willebrand disease are essential for optimal management. The von Willebrand factor multimers analysis is in the phenotypic classification, especially in discrepant cases, an integral part of the diagnostic process. The aim of this study was to evaluate the performance of a new Hydragel 11VWF multimer assay (H11VW). Results: Comparison study did not reveal any significant difference between H5VW and H11VW. The assessment of within-subject results, using H5VW and H1… Show more
“…Several independent investigators have previously reported on the analytical performance evaluation of the new Sebia technique with either 5-gel and 11-gel formats (3,12,14,15,18,20,21,23). Details of analytical performance of the Sebia method are beyond the scope of our current study.…”
Section: Calculation Of Reference Intervalsmentioning
confidence: 99%
“…The new semi-automated VWF multimer technique can help in standardization (26): it helps to reduce the interlaboratory variability and the variability between different measurement runs. Densitometry could contribute to its standardization by offering a reproducible quantification and additional visualization of VWF multimer patterns and permitting a precise quantitative comparison of sample patterns with those of a reference plasma curve (20).…”
Section: Calculation Of Reference Intervalsmentioning
Background: von Willebrand factor (VWF) multimers (VWF:MM) methodologies are technically difficult, laborious, time consuming, non-standardized and results vary between laboratories. A new semi automated VWF:MM assay is available for routine use (Sebia). Due to lack of reference values for VWF:MM fractions, results interpretation can be challenging in some cases. The aim of this study was to determine reference intervals for low molecular weight (LMWM), intermediate molecular weight (IMWM) and high molecular weight (HMWM) multimers. Methods: By the international cooperation initiated between 4 countries (Estonia, Latvia, France, and USA) 131 samples of relatively healthy individuals were analyzed for VWF:MM (in total 51 males and 80 non-pregnant females aged 17-69 years). Reference intervals were calculated according to CLSI C28-A3 standard. Results: The proposed reference intervals for VWF:MM were calculated for LMWM 10.4-22.5%, IMWM 22.6-37.6%, HMWM 45.6-66.6%. Age related differences were seen in IMWM and HMWM (p<0.001 and 0.038). There was no gender related difference observed. Geographically LMWM results of France were different from the other regions (p<0.05). Conclusions: Quantification of VWF:MM fractions, in addition to qualitative assessment of VWF:MM patterns, has the potential to aid in differential diagnosis of von Willebrand disease (VWD) subtypes. The reference values calculated in this study can be used in future research to establish clinical decision limits.
“…Several independent investigators have previously reported on the analytical performance evaluation of the new Sebia technique with either 5-gel and 11-gel formats (3,12,14,15,18,20,21,23). Details of analytical performance of the Sebia method are beyond the scope of our current study.…”
Section: Calculation Of Reference Intervalsmentioning
confidence: 99%
“…The new semi-automated VWF multimer technique can help in standardization (26): it helps to reduce the interlaboratory variability and the variability between different measurement runs. Densitometry could contribute to its standardization by offering a reproducible quantification and additional visualization of VWF multimer patterns and permitting a precise quantitative comparison of sample patterns with those of a reference plasma curve (20).…”
Section: Calculation Of Reference Intervalsmentioning
Background: von Willebrand factor (VWF) multimers (VWF:MM) methodologies are technically difficult, laborious, time consuming, non-standardized and results vary between laboratories. A new semi automated VWF:MM assay is available for routine use (Sebia). Due to lack of reference values for VWF:MM fractions, results interpretation can be challenging in some cases. The aim of this study was to determine reference intervals for low molecular weight (LMWM), intermediate molecular weight (IMWM) and high molecular weight (HMWM) multimers. Methods: By the international cooperation initiated between 4 countries (Estonia, Latvia, France, and USA) 131 samples of relatively healthy individuals were analyzed for VWF:MM (in total 51 males and 80 non-pregnant females aged 17-69 years). Reference intervals were calculated according to CLSI C28-A3 standard. Results: The proposed reference intervals for VWF:MM were calculated for LMWM 10.4-22.5%, IMWM 22.6-37.6%, HMWM 45.6-66.6%. Age related differences were seen in IMWM and HMWM (p<0.001 and 0.038). There was no gender related difference observed. Geographically LMWM results of France were different from the other regions (p<0.05). Conclusions: Quantification of VWF:MM fractions, in addition to qualitative assessment of VWF:MM patterns, has the potential to aid in differential diagnosis of von Willebrand disease (VWD) subtypes. The reference values calculated in this study can be used in future research to establish clinical decision limits.
“…Before 2016, it was not possible to confirm a suspicion of AVWS in Estonia because of a limitation of available laboratory VWF assays, while, since 2016, all VWF-related screening assays have been available to clinicians 5 and, recently, a semiautomated VWF multimer assay has been incorporated into routine clinical practice at the North Estonia Medical Centre (NEMC). 6,7 Here, we describe the clinical and laboratory data of seven patients diagnosed with AVWS at NEMC.…”
Objectives Acquired von Willebrand syndrome (AVWS) is a rare and frequently underdiagnosed bleeding disorder with an unknown prevalence. The diagnosis of AVWS is made based on laboratory investigations and the presence of clinical symptoms. Evaluation and management of affected patients are complex due to the need for multiple laboratory assays.
Materials and Methods Here, we describe the clinical and laboratory data of seven patients with a diagnosis of AVWS. All patients met the criteria for AVWS based on laboratory findings, bleeding symptoms, and the absence of any previous history of a bleeding disorder.
Results In all cases, the laboratory findings, lack of bleeding anamnesis, and family history suggested the presence of AVWS. Von Willebrand factor multimeric analysis showed decreased high-molecular weight (HMW) multimers in six cases. Patients with lower HMW multimers experienced more severe bleeding complications.
Conclusions The diagnosis of AVWS is complex and requires extensive laboratory evaluation. Interdisciplinary collaboration and complex laboratory evaluations are of paramount importance for the early recognition of AVWS and optimal AVWS diagnosis as well as successful clinical management.
“…These assays are labour-intensive, timeconsuming, nonstandardized and may miss subtle changes. [7][8][9][10] These conventional methods have high error rates in proficiency scheme participation as reported for example by the External Quality Control of Diagnostic Assays and Tests (ECAT) scheme. 11 A new semi-automated VWF multimer electrophoresis assay, the Hydragel 5 VWF multimers kit ® (Sebia ® ), enables VWF multimer analysis and produces multimer patterns that can be visually inspected or viewed using densitometry.…”
mentioning
confidence: 99%
“…This assay is more standardized, less labour-intensive and has a shorter turnaround time when compared with conventional electrophoretic assays. [7][8][9][10] The current study aimed to verify the performance of the Hydragel 5 VWF multimers kit ® by comparing results obtained with the new assay to those obtained with existing manual electrophoretic multimer assay. 1).…”
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