摘要 目的： 了解原发性骨髓纤维化(IMF)临床特点及疾病的演变情况，方法： 回顾性分析了25例IMF的临床及实验室检查资料。结果： 52%(13/25)的患者曾被误诊：72%(18/25)确诊时年龄超过50岁。81%(17/21)脾肿大，巨脾者占57%(12/21)，4例初诊时已切脾。72%(18/25)肝肿大，80%(20/25)外周血出现幼稚粒细胞，72%(18/25)外周血出现幼红细胞。病期中有2例IMF转变为急性白血病。初诊时骨髓纤维化Ⅱ期及Ⅲ期患者的外周血细胞数量及诊断后的中位生存期无显著性差异(P>0.05)。结论： IMF易被误诊，纤维化的过程在部分病人可能是非进行性的。目前治疗效果差，预后不良。

    中国图书资料分类法分类号 R551.3

CLINICAL STUDY OF TWENTY-FIVE CASES OF IDIOPATHIC MYELOFIBRISIS

Zhou Jiansheng

    (Department of Hematology,the First Affiliated Hospital of Guangxi Medical University, Nanning 530021)

Abstract Objective: To understand the clinical characteristic and thedisease course of idiopathic myelofibrosis (IMF).Mathods: To analyze theclinical and laboratory material of 25 cases of IMF, retrospectively.Results: Thirteenof twenty-five patients were initially diagnosed mistakenly.72% of the patients (18/25)were over the age of fifty at diagnosis. splenomegaly and hepatomegaly occurred in 17 ofthe 21 patients and 18 of the 25 patients respectively. Four patients had beensplenectomized before diagnosis. Immature granulocytes and nucleated red blood cells inthe peripheral blood were found in 20 and 17 of the 25 patients respectively. The quantityof peripheral blood cells and the median survival durations were not significantlydifferent between patients in the Ⅱand ⅢBM stage. Two cases developed blasttransformations in their disease course.Conclusion: IMF is easilymistakenly diagnosed. The fibrosis procedures may be not progressive in some patients. Theprognosis of IMF is poor.

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