TY - JOUR
T1 - Semi-automated von Willebrand factor multimer assay for von Willebrand disease
T2 - Further validation, benefits and limitations
AU - Oliver, Susan
AU - Vanniasinkam, Thiru
AU - Mohammed, Soma
AU - Vong, Ronny
AU - Favaloro, Emmanuel J
PY - 2019/12
Y1 - 2019/12
N2 -
Introduction
Accurate diagnosis of von Willebrand disease (VWD)
enables effective patient management. von Willebrand factor (VWF)
multimer analysis provides useful information regarding VWF multimer
structure, thereby aiding VWD subtyping and management; however,
historically technically challenging assays have had limited utility.
This study evaluates the Sebia Hydrasys Hydragel-11 semi-automated VWF
multimer assay and further validates the Hydragel-5 gel system, as
primarily pertaining to VWD diagnostics and monitoring of therapy.
Methods
Provisionally diagnosed (via a reference assay test
panel) archived patient samples and prospective test patient samples,
including those undergoing desmopressin trial or therapy monitoring,
along with commercial and in-house control material and various external
quality assessment (EQA) samples, were analysed. VWF multimers were
evaluated for presence, loss or partial loss of high molecular weight
(HMWM) and intermediate molecular weight (IMWM) multimers by both visual
inspection and densitometric scanning, and comparison with reference
assay results.
Results
All anticipated multimer patterns were reproduced, with
patients generally showing multimer profiles matching expected patterns
according to VWD type based on reference test panel ‘diagnosis’.
Occasional discrepancies were resolved by retesting. The increase in
plasma VWF following desmopressin therapy was also clearly demonstrated.
Multimer profiles of EQA samples complemented reference test panel
results and matched EQA targets. There were some ‘technical’ limitations
noted.
Conclusion
This easy to use, standardised, semi-automated multimer
analysis system can demonstrate the multimer profile of VWD patients,
thus representing an additional laboratory tool for improved diagnosis,
thereby facilitating appropriate patient management.
AB -
Introduction
Accurate diagnosis of von Willebrand disease (VWD)
enables effective patient management. von Willebrand factor (VWF)
multimer analysis provides useful information regarding VWF multimer
structure, thereby aiding VWD subtyping and management; however,
historically technically challenging assays have had limited utility.
This study evaluates the Sebia Hydrasys Hydragel-11 semi-automated VWF
multimer assay and further validates the Hydragel-5 gel system, as
primarily pertaining to VWD diagnostics and monitoring of therapy.
Methods
Provisionally diagnosed (via a reference assay test
panel) archived patient samples and prospective test patient samples,
including those undergoing desmopressin trial or therapy monitoring,
along with commercial and in-house control material and various external
quality assessment (EQA) samples, were analysed. VWF multimers were
evaluated for presence, loss or partial loss of high molecular weight
(HMWM) and intermediate molecular weight (IMWM) multimers by both visual
inspection and densitometric scanning, and comparison with reference
assay results.
Results
All anticipated multimer patterns were reproduced, with
patients generally showing multimer profiles matching expected patterns
according to VWD type based on reference test panel ‘diagnosis’.
Occasional discrepancies were resolved by retesting. The increase in
plasma VWF following desmopressin therapy was also clearly demonstrated.
Multimer profiles of EQA samples complemented reference test panel
results and matched EQA targets. There were some ‘technical’ limitations
noted.
Conclusion
This easy to use, standardised, semi-automated multimer
analysis system can demonstrate the multimer profile of VWD patients,
thus representing an additional laboratory tool for improved diagnosis,
thereby facilitating appropriate patient management.
KW - desmopressin
KW - Hydragel
KW - Multimers
KW - von Willebrand disease
KW - von Willebrand factor
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U2 - 10.1111/ijlh.13107
DO - 10.1111/ijlh.13107
M3 - Article
C2 - 31508897
AN - SCOPUS:85072184961
SN - 1751-5521
VL - 41
SP - 762
EP - 771
JO - International Journal of Laboratory Hematology
JF - International Journal of Laboratory Hematology
IS - 6
ER -