Clinical characteristics of 12 patients with primary myelofibrosis and liver cirrhosis
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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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Key words:
- primary myelofibrosis /
- liver cirrhosis /
- splenomegaly /
- signs and symptoms
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