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Thromb Haemost 2022; 122(10): 1673-1682
DOI: 10.1055/a-1806-9972
Cellular Haemostasis and Platelets

HemosIL VWF:GPIbR Assay Has a Greater Sensitivity than VWF:RCo Technique to Detect Acquired von Willebrand Syndrome in Myeloproliferative Neoplasms

Pierre Laporte   *
1   Bordeaux University Hospital, Laboratory of Hematology, Pessac, France
,
Marie Tuffigo   *
2   Angers University Hospital, Laboratory of Hematology, Angers, France
,
Anne Ryman
1   Bordeaux University Hospital, Laboratory of Hematology, Pessac, France
,
Mathieu Fiore
1   Bordeaux University Hospital, Laboratory of Hematology, Pessac, France
,
Etienne Rivière
3   Bordeaux University Hospital, Internal Medicine, Pessac, France
4   University of Bordeaux, UMR1034, Inserm, Biology of Cardiovascular Diseases, Pessac, France
,
Chloé James
1   Bordeaux University Hospital, Laboratory of Hematology, Pessac, France
4   University of Bordeaux, UMR1034, Inserm, Biology of Cardiovascular Diseases, Pessac, France
,
Alexandre Guy 
1   Bordeaux University Hospital, Laboratory of Hematology, Pessac, France
4   University of Bordeaux, UMR1034, Inserm, Biology of Cardiovascular Diseases, Pessac, France
› Author Affiliations Funding This study was supported by research grants from INSERM, the Fondation Bettencourt Schueller.
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Abstract

Background Acquired von Willebrand syndrome (AVWS) is frequent in patients with myeloproliferative neoplasms (MPNs). For von Willebrand factor (VWF) functional evaluation, ristocetin cofactor activity by aggregometry (VWF:RCo) is considered the gold standard but has limitations, and automated activity measurement has been developed such as the HemosIL VWF:RCo Werfen with particle agglutination (VWF:GPIbR).

Objectives To evaluate the performance of VWF:GPIbR with HemosIL VWF:RCo Werfen (VWF:GPIbR) versus VWF:RCo in patients with thrombocytosis in the context of MPNs (T-MPNs) and in patients with secondary thrombocytosis (ST).

Methods MPN patients with thrombocytosis >450 G/L (T-MPNs) were compared with patients with ST due to inflammation or iron deficiency. VWF activity (VWF:Act) was analyzed using VWF:RCo or VWF:GPIbR. VWF analysis was completed by analysis of VWF multimers and VWF collagen binding (CB) assay (VWF:CB).

Results A total of 33 T-MPNs and 18 ST patients were included. Compared with aggregometry, evaluation of VWF:Act by VWF:GPIbR led to lower values in T-MPN patients, but also in ST patients. Interestingly, although the VWF:RCo/VWF:Ag ratio did not reveal differences between T-MPNs and ST patients, the VWF:GPIbR/VWF:Ag ratio analysis allowed us to suspect AVWS only in T-MPN patients. Using the distribution of VWF multimer analysis and VWF:CB, we here demonstrated that VWF:GPIbR allows AVWS diagnosis in nine T-MPNs as opposed to aggregometry.

Conclusion Evaluation of VWF:Act using VWF:GPIbR has a greater sensitivity compared with aggregometry to detect AVWS in T-MPN patients.

Keywords

acquired von Willebrand syndrome - myeloproliferative neoplasms - thrombocytosis

Author Contributions

P.L., M.T., A.R., and M.F, contributed to designing and performing experiments, analysis and interpretation of data, and preparing the manuscript. A.R. and M.F. contributed to performing experiments, analysis and interpretation of data, and preparing the manuscript. E.R. contributed to providing patients' blood samples and preparing the manuscript. C.J. and A.G. contributed to supervising the study, designing experiments, analysis and interpretation of data, and preparing the manuscript. All authors discussed the results and commented on the manuscript.


* These authors contributed equally to this work.


Supplementary Material



Publication History

Received: 23 November 2021

Accepted: 22 March 2022

Accepted Manuscript online:
23 March 2022

Article published online:
28 June 2022

© 2022. Thieme. All rights reserved.

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