Platelet function analyzer (PFA-100) in patients with mild to moderate bleeding disorders and bleeding disorder of unknown cause.

IF 5.5 2区医学 Q1 HEMATOLOGY Journal of Thrombosis and Haemostasis Pub Date : 2025-02-05 DOI:10.1016/j.jtha.2025.01.011

Dino Mehic, Bernhard Eichinger, Tim Dreier, Alexander Tolios, Beate Eichelberger, Alexandra Kaider, Cihan Ay, Ingrid Pabinger, Johanna Gebhart

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引用次数: 0

Abstract

Background: The sensitivity of the platelet function analyzer (PFA-100) was shown to be high for the detection of von Willebrand disease (VWD), but limited for platelet function defects (PFD). In patients with bleeding disorder of unknown cause (BDUC), however, data on the usefulness of PFA-100 is lacking.

Methods: PFA-100 closure times (CTs) were measured with collagen-epinephrine [PFA (EPI)] and collagen-adenosine diphosphate [PFA (ADP)] cartridges in 818 patients with mild bleeding disorders from the Vienna Bleeding Biobank. Patients on anticoagulation or antiplatelet therapy or thrombocytopenic patients were not included.

Results: Only 2% of the 532 BDUC patients had prolonged CTs in PFA (EPI) and PFA (ADP), and 64% in either PFA (EPI) or PFA (ADP). In total, 34% of BDUC patients did not have prolonged CTs in PFA (EPI) or PFA (ADP). These rates were similar to patients with coagulation factor deficiencies (n=27). The rate of pathological CTs was significantly higher in patients with VWD (n=79) and, although less pronounced, in PFD (n=180). In 15/18 (83%) VWD patients with VWF:Ag and/or VWF:RCo levels <30 IU/dL, the PFA-100 was prolonged in both cartridges. No association of the PFA-100 with the bleeding severity was observed in BDUC patients. However, prolonged CTs were associated with higher age, lower hematocrit, lower VWF:Ag or VWF:RCo levels, lower platelet counts, and higher fibrinogen levels in BDUC patients.

Conclusion: We could not confirm a diagnostic utility for the PFA-100 in MBD patients, and specifically BDUC. No association between PFA-100 results and bleeding severity was observed in BDUC patients.

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来源期刊

Journal of Thrombosis and Haemostasis 医学-外周血管病

CiteScore

24.30

自引率

3.80%

发文量

321

审稿时长

1 months

期刊介绍： The Journal of Thrombosis and Haemostasis (JTH) serves as the official journal of the International Society on Thrombosis and Haemostasis. It is dedicated to advancing science related to thrombosis, bleeding disorders, and vascular biology through the dissemination and exchange of information and ideas within the global research community. Types of Publications: The journal publishes a variety of content, including: Original research reports State-of-the-art reviews Brief reports Case reports Invited commentaries on publications in the Journal Forum articles Correspondence Announcements Scope of Contributions: Editors invite contributions from both fundamental and clinical domains. These include: Basic manuscripts on blood coagulation and fibrinolysis Studies on proteins and reactions related to thrombosis and haemostasis Research on blood platelets and their interactions with other biological systems, such as the vessel wall, blood cells, and invading organisms Clinical manuscripts covering various topics including venous thrombosis, arterial disease, hemophilia, bleeding disorders, and platelet diseases Clinical manuscripts may encompass etiology, diagnostics, prognosis, prevention, and treatment strategies.