目的　探讨儿童原发性血小板增多症的诊断和治疗。方法　回顾性分析8例儿童原发性血小板增多症患儿的临床资料。结果　2010年1月至2015年6月门诊诊治8例儿童原发性血小板增多症患儿，年龄10个月~12岁；6例患儿显示JAK2V617F突变，2例无突变；采用羟基脲或α干扰素治疗，均获得缓解。结论　原发性血小板增多症在儿童时期是罕见疾病，但有增多趋势；需要更多的病例比较JAK2V617F突变阳性和阴性患儿临床表现和预后的差异。

Objective　To explore the diagnosis and treatment of essential thrombocythemia in children. Methods　The clinical data from 8 children with essential thrombocythemia were retrospectively analyzed. Results　From January 2010 to June 2015, there were 8 children diagnosed essential thrombocythemia in clinic. One child was only 10 months old. Among them, 6 cases had JAK2V617F mutation, 2 cases had no mutation. All children were treated with hydroxyurea or interferon-α, and achieve remission. Conclusion　Essential thrombocythemia is a rare disease in childhood with a growing trend. More children with essential thrombocythemia should be enrolled for comparison of clinical manifestations and prognosis between JAK2V617F mutation positive and negative cases.