原发性骨髓纤维化的临床研究

CLINICAL STUDY OF TWENTY FIVE CASES OF IDIOPATHIC MYELOFIBRISIS

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。

Objective:To understand the clinical characteristic and the disease course of idiopathic myelofibrosis (IMF) Mathods:To analyze the clinical and laboratory material of 25 cases of IMF, retrospectively Results:Thirteen of 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 of the 21 patients and 18 of the 25 patients respectively Four patients had been splenectomized before diagnosis Immature granulocytes and nucleated red blood cells in the peripheral blood were found in 20 and 17 of the 25 patients respectively The quantity of peripheral blood cells and the median survival durations were not significantly different between patients in the Ⅱand ⅢBM stage Two cases developed blast transformations in their disease course Conclusion:IMF is easily mistakenly diagnosed The fibrosis procedures may be not progressive in some patients The prognosis of IMF is poor