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原发性血小板增多症的临床和预后特征:2001 年 WHO 标准与 2008/2016 年 WHO 标准在一个大型单中心队列中的比较。

Clinical and Prognostic Features of Essential Thrombocythemia: Comparison of 2001 WHO Versus 2008/2016 WHO Criteria in a Large Single-center Cohort.

机构信息

Department of Translational and Precision Medicine, University "Sapienza" of Rome, Rome, Italy.

Department of Translational and Precision Medicine, University "Sapienza" of Rome, Rome, Italy.

出版信息

Clin Lymphoma Myeloma Leuk. 2021 Apr;21(4):e328-e333. doi: 10.1016/j.clml.2020.11.003. Epub 2020 Nov 6.

DOI:10.1016/j.clml.2020.11.003
PMID:33342728
Abstract

BACKGROUND

According to 2008/2016 classification of the World Health Organization (WHO), a platelet (PLT) count ≥ 450 × 10/L, reduced from the previously published WHO 2001 indicated level ≥ 600 × 10/L, was considered the new PLT threshold for the diagnosis of essential thrombocythemia (ET).

PATIENTS AND METHODS

To validate this important diagnostic change in a setting of current clinical practice, we retrospectively analyzed clinical and hematologic features at diagnosis and during follow-up of 162 patients with ET, diagnosed in our center from January 2008 to December 2017. We subdivided patients according to PLT value at baseline into Group A (PLT ≥ 600 × 10/L) (124 patients; 76.5%) and Group B (PLT ≥ 450 × 10/L < 600 × 10/L) (38 patients; 23.5%).

RESULTS

Among clinical features, only the median value of leukocytes (P < .001) was significantly higher in Group A. Cytostatic treatment was administered in 103 patients, with a significantly higher rate in patients of group A (P < .001). After a median follow-up of 42.4 months (interquartile range, 22.1-70.6 months), 8 thrombotic events were recorded in the entire cohort, without differences between the 2 groups (P = .336). The 5-year overall survival (OS) of the entire cohort was 96.9% (95% confidence interval, 92.6%-100%), without differences between the 2 groups (P = .255).

CONCLUSIONS

Our data indicate a substantial homogeneity among patients with ET regardless of the PLT count at diagnosis, thus confirming the usefulness of the 2008/2016 WHO diagnostic criteria.

摘要

背景

根据世界卫生组织(WHO)2008/2016 年的分类,血小板(PLT)计数≥450×10/L,较之前发表的 WHO 2001 年规定的≥600×10/L有所降低,被认为是诊断原发性血小板增多症(ET)的新 PLT 阈值。

患者和方法

为了在当前临床实践环境中验证这一重要的诊断变化,我们回顾性分析了 2008 年 1 月至 2017 年 12 月期间在我院诊断为 ET 的 162 例患者的诊断时和随访期间的临床和血液学特征。我们根据基线时的 PLT 值将患者分为 A 组(PLT≥600×10/L)(124 例;76.5%)和 B 组(PLT≥450×10/L<600×10/L)(38 例;23.5%)。

结果

在临床特征中,仅 A 组白细胞的中位数(P<0.001)显著更高。103 例患者接受了细胞抑制治疗,A 组患者的比例显著更高(P<0.001)。在中位随访 42.4 个月(四分位间距,22.1-70.6 个月)后,整个队列中记录到 8 例血栓事件,两组间无差异(P=0.336)。整个队列的 5 年总生存率(OS)为 96.9%(95%置信区间,92.6%-100%),两组间无差异(P=0.255)。

结论

我们的数据表明,无论诊断时的 PLT 计数如何,ET 患者之间存在实质性的同质性,从而证实了 2008/2016 年 WHO 诊断标准的有用性。

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Clinical and Prognostic Features of Essential Thrombocythemia: Comparison of 2001 WHO Versus 2008/2016 WHO Criteria in a Large Single-center Cohort.原发性血小板增多症的临床和预后特征:2001 年 WHO 标准与 2008/2016 年 WHO 标准在一个大型单中心队列中的比较。
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