我们的免疫比浊法 VWF:GPIbR 检测在骨髓增殖性肿瘤中获得性血管性血友病的敏感性高于 VWF:RCo 技术。

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

机构信息

Bordeaux University Hospital, Laboratory of Hematology, Pessac, France.

Angers University Hospital, Laboratory of Hematology, Angers, France.

出版信息

Thromb Haemost. 2022 Oct;122(10):1673-1682. doi: 10.1055/a-1806-9972. Epub 2022 Mar 23.

DOI:10.1055/a-1806-9972

PMID:35322397

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.

摘要

背景

获得性血管性血友病（AVWS）在骨髓增殖性肿瘤（MPN）患者中很常见。对于血管性血友病因子（VWF）功能评估，聚集法测定瑞斯托霉素辅因子活性（VWF：RCo）被认为是金标准，但存在局限性，并且已经开发了自动化活性测量方法，例如使用颗粒聚集的 HemosIL VWF：RCo Werfen（VWF：GPIbR）。

目的

评估 HemosIL VWF：RCo Werfen（VWF：GPIbR）与 VWF：RCo 在 MPN 伴发血小板增多症（T-MPNs）和继发性血小板增多症（ST）患者中的性能。

方法

将血小板计数 >450 G/L 的 MPN 患者（T-MPNs）与因炎症或铁缺乏引起的 ST 患者进行比较。使用 VWF：RCo 或 VWF：GPIbR 分析 VWF 活性（VWF：Act）。通过 VWF 多聚体分析和 VWF 胶原结合（CB）测定（VWF：CB）完成 VWF 分析。

结果

共纳入 33 例 T-MPNs 和 18 例 ST 患者。与聚集法相比，使用 VWF：GPIbR 评估 VWF：Act 导致 T-MPN 患者和 ST 患者的 VWF：Act 值降低。有趣的是，尽管 VWF：RCo/VWF：Ag 比值在 T-MPNs 和 ST 患者之间没有差异，但 VWF：GPIbR/VWF：Ag 比值分析仅允许我们怀疑 T-MPN 患者存在 AVWS。通过 VWF 多聚体分析和 VWF：CB 的分布，我们在这里证明 VWF：GPIbR 可以在 9 例 T-MPN 中诊断 AVWS，而不是聚集法。