Labar Boris, Kusec Rajko, Jaksić Branimir, Skare-Librenjak Ljubica, Nacinović-Duletić Antica, Petricević-Sincić Jasminka, Carzavec Dubravka, Coha Bozena, Gverić-Krecak Velka, Corović Elizabeta, Lang Nada, Lojen-Nemet Zlata, Babok-Flegarić Renata
Klinika za unutrasnje bolesti, Medicinski fakultet Sveucilista u Zagrebu, KBC Zagreb.
Lijec Vjesn. 2010 Nov-Dec;132(11-12):333-9.
Essential thrombocythemia (ET) is a clonal myeloproliferative neoplasm. Croatian Cooperative Group for hematologic disorders, KROHEM proposes the diagnostic and treatment guidelines for ET. Diagnosis of ET is based on the criteria and classification of World Health Organization (WHO). The level of treatment recommendation is based on the UpToDate (web based medical community database) criteria. For ET diagnosis it is mandatory to show sustained increased number of platelets with typical histomorphological changes of megakaryopoiesis in bone marrow. Secondary thrombocytosis and other chronic myeloproliferative neoplasms have to be excluded. Therapy is based on risk factors for ET. The risk factors are number of platelets, patient's age, and the risk levels for thrombosis and bleeding. Patients with low risk (age < 60 years and platelets < 1000 x 10(9)/L) arw not candidates for therapy. In younger group of patients with platelets between 1000 and 1500 x 10(9)/L or more than 1500 x 10(9)/L treatment with anagrelide or hydroxyurea is recommended respectively. In high risk patients hydroxyurea is the first line treatment. Anagrelide is indicated in these patients in the absence of treatment response. Alpha-interferon is recommended for pregnant women with ET and high platelet counts.
原发性血小板增多症(ET)是一种克隆性骨髓增殖性肿瘤。克罗地亚血液疾病合作组织KROHEM提出了ET的诊断和治疗指南。ET的诊断基于世界卫生组织(WHO)的标准和分类。治疗建议水平基于UpToDate(基于网络的医学社区数据库)标准。对于ET诊断,必须显示血小板数量持续增加,并伴有骨髓巨核细胞生成的典型组织形态学变化。必须排除继发性血小板增多症和其他慢性骨髓增殖性肿瘤。治疗基于ET的危险因素。危险因素包括血小板数量、患者年龄以及血栓形成和出血的风险水平。低风险患者(年龄<60岁且血小板<1000×10⁹/L)不适合治疗。对于血小板在1000至1500×10⁹/L之间或超过1500×10⁹/L的年轻患者组,分别建议使用阿那格雷或羟基脲治疗。高风险患者中,羟基脲是一线治疗药物。在这些患者中,如果没有治疗反应,则使用阿那格雷。对于血小板计数高的ET孕妇,建议使用α干扰素。