Accurso Vincenzo, Santoro Marco, Mancuso Salvatrice, Napolitano Mariasanta, Carlisi Melania, Mattana Marta, Russo Chiara, Di Stefano Alessandro, Sirocchi Davide, Siragusa Sergio
Hematology Division University Hospital Policlinico "Paolo Giaccone", Palermo, Italy.
Department of Surgical, Oncological and Stomatological Disciplines, University of Palermo, Palermo, Italy.
Clin Med Insights Blood Disord. 2020 Dec 28;13:2634853520978210. doi: 10.1177/2634853520978210. eCollection 2020.
The Essential Thrombocythemia is a Chronic Philadelphia-negative Myeloproliferative Neoplasm characterized by a survival curve that is only slightly worse than that of age- and sex-adjusted healthy population. The criteria for diagnosis were reviewed in 2016 by WHO. The incidence varies from 0.2 to 2.5:100 000 people per year, with a prevalence of 38 to 57 cases per 100 000 people. The main characteristics of ET are the marked thrombocytosis and the high frequency of thrombosis. The spectrum of symptoms is quite wide, but fatigue results to be the most frequent. Thrombosis is frequently observed, often occurring before or at the time of diagnosis. The classification of thrombotic risk has undergone several revisions. Recently, the revised-IPSET-t has distinguished 4 risk classes, from very low risk to high risk. Driver mutations seem to influence thrombotic risk and prognosis, while the role of sub-driver mutations still remains uncertain. Antiplatelet therapy is recommended in all patients aged ⩾ 60 years and in those with a positive history of thrombosis or with cardiovascular risk factors, while cytoreductive therapy with hydroxyurea or interferon is reserved for high-risk patients.
原发性血小板增多症是一种慢性费城染色体阴性骨髓增殖性肿瘤，其生存曲线仅略差于年龄和性别校正后的健康人群。世界卫生组织在2016年对诊断标准进行了审查。发病率为每年0.2至2.5：10万人口，患病率为每10万人38至57例。原发性血小板增多症的主要特征是显著的血小板增多和血栓形成的高频率。症状范围相当广泛，但疲劳是最常见的。经常观察到血栓形成，通常在诊断之前或之时发生。血栓形成风险的分类已经历了几次修订。最近，修订后的IPSET-t区分了4个风险类别，从极低风险到高风险。驱动基因突变似乎影响血栓形成风险和预后，而亚驱动基因突变的作用仍不确定。所有年龄≥60岁的患者以及有血栓形成阳性病史或有心血管危险因素的患者建议使用抗血小板治疗，而羟基脲或干扰素的细胞减灭治疗则保留给高危患者。
