原发性血小板增多症、真性红细胞增多症和慢性特发性骨髓纤维化中骨髓纤维化的发生率：对连续骨髓活检的回顾性评估

The incidence of myelofibrosis in essential thrombocythaemia, polycythaemia vera and chronic idiopathic myelofibrosis: a retrospective evaluation of sequential bone marrow biopsies.

作者信息

Kreft Andreas, Büche Guntram, Ghalibafian Maryam, Buhr Thomas, Fischer Thomas, Kirkpatrick Charles James

机构信息

Institut fü Pathologie, Klinikum Johannes-Gutenberg-Universitä Mainz, Mainz, Deutschland.

出版信息

Acta Haematol. 2005;113(2):137-43. doi: 10.1159/000083452.

DOI:10.1159/000083452

PMID:15802893

Abstract

The incidence of myelofibrosis (MF) among the three major Philadelphia chromosome-negative chronic myeloproliferative disorders, i.e. essential thrombocythaemia (ET), polycythaemia vera (PV) and chronic idiopathic myelofibrosis (CIMF), is not well documented since the diagnostic criteria have recently been redefined by the WHO. Therefore we performed a retrospective analysis of follow-up biopsies of 275 patients with ET, PV and CIMF according to the WHO classification of chronic myeloproliferative disorders. In the diagnostic bone marrow biopsies, MF was observed in 57 of the 136 CIMF patients (42%), 4 of the 73 PV patients (5%) and none of the 66 patients with ET. Within a median observation time of 2.9 years, 34 of the 79 patients with CIMF (43%), 13 of the 69 patients with PV (19%) and 1 of the 66 patients with ET (1.5%)--each initially without MF--developed MF regardless of myelosuppressive therapy.

摘要

在三种主要的费城染色体阴性慢性骨髓增殖性疾病，即原发性血小板增多症（ET）、真性红细胞增多症（PV）和慢性特发性骨髓纤维化（CIMF）中，骨髓纤维化（MF）的发病率尚无充分记录，因为世界卫生组织（WHO）最近重新定义了诊断标准。因此，我们根据WHO对慢性骨髓增殖性疾病的分类，对275例ET、PV和CIMF患者的随访活检进行了回顾性分析。在诊断性骨髓活检中，136例CIMF患者中有57例（42%）观察到MF，73例PV患者中有4例（5%）观察到MF，66例ET患者中均未观察到MF。在中位观察时间2.9年期间，79例最初无MF的CIMF患者中有34例（43%）、69例PV患者中有13例（19%）以及66例ET患者中有1例（1.5%）——无论是否接受骨髓抑制治疗——均发展为MF。