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12例原发性骨髓纤维化合并肝硬化患者的临床特征分析

王宣 何婷婷 张宁 周超 王葽 白云峰 张晶晶 付双楠 梁肖肖 李昕 宫嫚

引用本文:
Citation:

12例原发性骨髓纤维化合并肝硬化患者的临床特征分析

DOI: 10.3969/j.issn.1001-5256.2020.11.025
基金项目: 

国家科技重大专项(2018ZX10725506-002); 

详细信息
  • 中图分类号: R575.2;R551.3

Clinical characteristics of 12 patients with primary myelofibrosis and liver cirrhosis

Research funding: 

 

  • 摘要:

    <正>原发性骨髓纤维化(primary myelofibrosis,PMF)是由异常造血干细胞的克隆性增殖,导致进行性骨髓纤维化的一种骨髓增殖性肿瘤[1]。有研究[2]发现部分骨髓纤维化患者可出现肝脏硬度值升高。近年来,亦有研究者对PMF合并肝硬化、门静脉高压、消化道出血等进行过临床报道[3-4],但病例数较少。此外,PMF作为一种罕见病,常伴有脾大、门静脉高压、进行性血细胞减少及消化道出血等,与肝硬化表现较为相似,当该病合并肝硬化时,易出现漏诊、误诊。PMF可导致肝硬化,但其进展至肝硬化的间隔时间不详,且无相关临床报道。因此,通过对本院PMF合并肝硬化病例的总结,并结合文献复习,有助于更好地认识PMF与肝硬化之间的关系,对临床早期诊断,减少漏诊、误诊十分重要。

     

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