罕见病 疑难病

儿童原发性血小板增多症8 例临床分析

  • 梁辉
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  • 1. 上海交通大学医学院附属新华医院崇明分院(上海 202150);2. 上海交通大学医学院附属新华医院血液科(上海 200092)

收稿日期: 2015-11-15

  网络出版日期: 2015-11-15

Clinical analysis of eight cases of essential thrombocythemia in children

  • LIANG Hui
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  • 1. Chongming Branch, Xinhua Hospital Affiliate to Shanghai Jiaotong University School of Medicine, Shanghai 202150,China;2. Department of Hematology, Xinhua Hospital Affiliate to Shanghai Jiaotong University School of Medicine,Shanghai 200092,China

Received date: 2015-11-15

  Online published: 2015-11-15

摘要

 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。

本文引用格式

梁辉 . 儿童原发性血小板增多症8 例临床分析[J]. 临床儿科杂志, 2015 , 33(11) : 979 . DOI: 10.3969 j.issn.1000-3606.2015.11.015

Abstract

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.
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