Thrombocytosis and bleeding in myeloproliferative neoplasms: exploring clinical diversity and risk of acquired von Willebrand syndrome—insights from a UK center

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-07-01 DOI:10.1016/j.rpth.2025.102954

Giulia Simini , Andrew Innes , Saravanan Vinayagam , Golzar Mobayen , Nilanthi Karawitage , Simone Claudiani , Zain Odho , Mike Laffan , Deepa J. Arachchillage

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Abstract

Background

Myeloproliferative neoplasms (MPNs) represent a group of blood disorders characterized by myeloid cell proliferation and an associated increased risk of thrombosis and bleeding. Platelet count may have a direct link to these complications.

Objectives

To share our MPN clinic’s experience with hemostatic testing and bleeding outcomes in patients with platelets ≥ 800 × 10⁹/L.

Methods

This was a single-center retrospective study from May 2022 to September 2024. Clinical characteristics, treatments, and bleeding events of patients with MPN or chronic myeloid leukemia were recorded. Laboratory assessments included full blood count, renal function, coagulation profiles, platelet function test, and von Willebrand factor (VWF) assays.

Results

A total of 39 patients were included, majority of whom received aspirin for thrombosis prevention (76%). The study found that bleeding complications occurred in 33% of patients, with mucocutaneous bleeding being the most common. There was a trend toward bleeding in patients on aspirin (P = .07). However, platelet count alone did not predict bleeding risk. While some patients showed abnormal VWF function, low VWF levels were not consistently associated with increased bleeding. Interestingly, we found moderate negative correlation between baseline VWF ristocetin/antigen and activated partial thromboplastin time (P = .02; r = −.37) and prothrombin time (P = .009, r = −.45), suggesting other potential coagulation imbalances associated with bleeding diathesis in MPNs. Post cytoreduction, there was a significant increase in mean VWF ristocetin/antigen ratio (P = .0009).

Conclusion

The study illustrates the limitations of relying solely on platelet counts to estimate bleeding risk in MPN patients. Assessment of VWF activity and careful selection of antithrombotic therapy were highlighted as important considerations.

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骨髓增生性肿瘤中的血小板增多和出血：探索获得性血管性血友病综合征的临床多样性和风险-来自英国中心的见解

骨髓增生性肿瘤（mpn）是一组血液疾病，其特征是骨髓细胞增殖和相关的血栓和出血风险增加。血小板计数可能与这些并发症有直接联系。目的分享我院MPN临床在血小板≥800 × 109/L患者止血试验及出血结局方面的经验。方法采用单中心回顾性研究，研究时间为2022年5月至2024年9月。记录MPN或慢性髓系白血病患者的临床特征、治疗方法和出血事件。实验室评估包括全血细胞计数、肾功能、凝血功能、血小板功能测试和血管性血友病因子（VWF）测定。结果共纳入39例患者，其中大部分患者（76%）使用阿司匹林预防血栓形成。研究发现33%的患者出现出血并发症，以皮肤粘膜出血最为常见。服用阿司匹林的患者有出血的趋势（P = .07）。然而，血小板计数单独不能预测出血风险。虽然一些患者表现出VWF功能异常，但低VWF水平并不总是与出血增加有关。有趣的是，我们发现基线VWF里斯托霉素/抗原与活化的部分凝血活素时间之间存在中度负相关(P = 0.02；r = - 0.37)和凝血酶原时间（P = 0.009, r = - 0.45），提示其他潜在凝血失衡与mpn出血素质相关。细胞减少后，平均VWF里斯托素/抗原比值显著升高（P = 0.0009）。结论本研究说明单纯依靠血小板计数来评估MPN患者出血风险的局限性。评估VWF活性和谨慎选择抗血栓治疗被强调为重要的考虑因素。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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