Angona Anna, Alvarez-Larrán Alberto, Bellosillo Beatriz, Martínez-Avilés Luz, Garcia-Pallarols Francesc, Longarón Raquel, Ancochea Àgueda, Besses Carles
Servicio de Hematología, Hospital del Mar-IMIM, Barcelona, España; Universitat Autònoma de Barcelona, Barcelona, España; Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España.
Grup de Recerca Clínica Aplicada en Neoplàsies Hematològiques, Hospital del Mar-IMIM, Barcelona, España; Servicio de Patología, Hospital del Mar-IMIM, Barcelona, España; Universitat Pompeu Fabra, Barcelona, España.
Med Clin (Barc). 2015 Mar 15;144(6):247-53. doi: 10.1016/j.medcli.2014.04.029. Epub 2014 Sep 3.
Two prognostic models to predict overall survival and thrombosis-free survival have been proposed: International Prognostic Score for Essential Thrombocythemia (IPSET) and IPSET-Thrombosis, respectively, based on age, leukocytes count, history of previous thrombosis, the presence of cardiovascular risk factors and the JAK2 mutational status. The aim of the present study was to assess the clinical and biological characteristics at diagnosis and during evolution in essential thrombocythemia (ET) patients as well as the factors associated with survival and thrombosis and the usefulness of these new prognostic models.
We have evaluated the clinical data and the mutation status of JAK2, MPL and calreticulin of 214 ET patients diagnosed in a single center between 1985 and 2012, classified according to classical risk stratification, IPSET and IPSET-Thrombosis.
With a median follow-up of 6.9 years, overall survival was not associated with any variable by multivariate analysis. Thrombotic history and leukocytes>10×10(9)/l were associated with thrombosis-free survival (TFS). In our series, IPSET prognostic systems of survival and thrombosis did not provide more clinically relevant information regarding the classic risk of thrombosis stratification.
Thrombotic history and leukocytosis>10×10(9)/l were significantly associated with lower TFS, while the prognostic IPSET-Thrombosis system did not provide more information than classical thrombotic risk assessment.
已提出两种预测总生存期和无血栓生存期的预后模型：分别是基于年龄、白细胞计数、既往血栓形成史、心血管危险因素的存在情况以及JAK2突变状态的原发性血小板增多症国际预后评分（IPSET）和IPSET-血栓形成模型。本研究的目的是评估原发性血小板增多症（ET）患者诊断时及病程中的临床和生物学特征，以及与生存和血栓形成相关的因素，还有这些新预后模型的实用性。
我们评估了1985年至2012年在单一中心诊断的214例ET患者的临床数据以及JAK2、MPL和钙网蛋白的突变状态，根据经典风险分层、IPSET和IPSET-血栓形成模型进行分类。
中位随访6.9年，多因素分析显示总生存期与任何变量均无关联。血栓形成史和白细胞计数>10×10⁹/L与无血栓生存期（TFS）相关。在我们的研究系列中，IPSET生存和血栓形成预后系统在经典血栓形成风险分层方面并未提供更多临床相关信息。
血栓形成史和白细胞增多>10×10⁹/L与较低的TFS显著相关，而IPSET-血栓形成预后系统相比经典血栓形成风险评估并未提供更多信息。
