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首页> 外文期刊>European Journal of Haematology >Neurological symptoms in essential thrombocythemia: impact of JAK2V617F mutation and response to therapy
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Neurological symptoms in essential thrombocythemia: impact of JAK2V617F mutation and response to therapy

机译：原发性血小板增多症的神经系统症状：JAK2V617F突变的影响和对治疗的反应

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Patients with essential thrombocythemia (ET) often suffer from neurological symptoms (NS) not ever resulting from previous thrombotic cerebral events (TCE). We reported NS occurred in 282 patients, in order to identify the factors influencing ET-related NS in the absence of TCE, and the response to therapy. Overall, 116 of 282 patients (41%) presented NS; 101 of them (87%) reported subjective transient and fluctuating NS, without concurrent TCE, which we defined as ET-related NS, by frequency: cephalalgia, chronic paresthesias, dizziness or hypotension, visual disturbances, and tinnitus. In univariate analysis, ET-related NS resulted more frequently in young people (P=0.017) and in females (P=0.025). We found a higher prevalence of JAK2V617F mutation in ET-related NS patients (P=0.021). In multivariate analysis, gender (P=0.024) and JAK2V617F mutation (P=0.041) remained significantly associated with the development of ET-related NS, with a risk of about four times higher for JAK2V617F-mutated patients (OR=3.75). Ninety-seven of 101 patients with ET-related NS received an antiplatelet (AP) agent at the time of NS, whereas only selected high-risk ET-related NS patients were treated with a cytoreductive drug, according to the published guidelines and similarly to patients without NS. We observed that only 32 of 97 (33%) patients with ET-related NS achieved a complete response after AP treatment. Among the 65 non-responder patients, 36 (55.4%) improved NS after the introduction of cytoreductive therapy; therefore, the addition of cytoreductive treatment should be considered in this setting.
机译：患有原发性血小板增多症（ET）的患者通常患有神经系统症状（NS），而以前从未出现过血栓性脑事件（TCE）。我们报告了282例患者发生NS，目的是确定在没有TCE的情况下影响ET相关NS的因素以及对治疗的反应。总体而言，282名患者中有116名（41％）出现了NS;他们中有101人（占87％）报告了主观的短暂性和波动性NS，没有并发的TCE，我们将其定义为与ET相关的NS，其发生频率包括：头痛，慢性感觉异常，头晕或低血压，视力障碍和耳鸣。在单变量分析中，与ET相关的NS在年轻人（P = 0.017）和女性（P = 0.025）中的发生率更高。我们发现与ET相关的NS患者中JAK2V617F突变的患病率更高（P = 0.021）。在多变量分析中，性别（P = 0.024）和JAK2V617F突变（P = 0.041）仍然与ET相关NS的发展密切相关，而JAK2V617F突变患者的风险大约高四倍（OR = 3.75）。根据公布的指南，在101例与ET相关的NS患者中，有97例在接受NS时接受了抗血小板（AP）药物治疗，而只有少数高危ET相关的NS患者接受了细胞减少药物治疗。没有NS的患者。我们观察到97例与ET相关的NS患者中只有32例（33％）在接受AP治疗后完全缓解。 65例无反应者中，有36例（55.4％）在采用减细胞疗法后改善了NS。因此，在这种情况下，应考虑加用减细胞治疗。

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