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巨核细胞增殖对骨髓纤维化演变的影响。186例慢性髓性白血病患者的组织学随访研究。

The impact of megakaryocyte proliferation of the evolution of myelofibrosis. Histological follow-up study in 186 patients with chronic myeloid leukaemia.

作者信息

Buhr T, Choritz H, Georgii A

机构信息

Pathologisches Institut, Medizinischen Hochschule Hannover, Federal Republic of Germany.

出版信息

Virchows Arch A Pathol Anat Histopathol. 1992;420(6):473-8. doi: 10.1007/BF01600251.

Abstract

A histological study on sequential bone marrow biopsies in patients with chronic myeloid leukaemia (CML) was performed. We wished to answer the question as to whether a different content of megakaryopoiesis in the bone marrow of CML patients has a prognostic significance for the development of myelofibrosis during the course of disease. In addition, the significance of possible changes in the quantity of megakaryopoiesis in this process was assessed. In 186 patients who had no fibre increase at first diagnosis, the rate of subsequent myelofibrosis varied from 19% for the common or granulocytic subtype (CML.CT) to 40% for patients with features of megakaryocytic increase (CML.MI). No significant differences were found either in the rapidity of progression to fibrosis or in the final rate of osteomyelosclerosis. Whereas in CML.MI most patients (75%) showed an increase of fibres only, this was accompanied by an additional increase of megakaryocytes in CML.CT, changing the histological pattern from CML.CT to .MI or .MP, respectively. The data therefore revealed a correlation between fibre increase and subtyping of CML as suggested by the Hannover classification of chronic myeloproliferative diseases. Subtypes of CML with megakaryocytic increase could be shown to present a "pre-myelofibrotic" stage of disease and may therefore be conceived as a particular pathway of acceleration.

摘要

对慢性髓性白血病(CML)患者进行了序贯骨髓活检的组织学研究。我们希望回答CML患者骨髓中巨核细胞生成的不同含量对于疾病过程中骨髓纤维化的发展是否具有预后意义这一问题。此外,还评估了在此过程中巨核细胞生成数量可能变化的意义。在186例初诊时无纤维增多的患者中,随后发生骨髓纤维化的比例从常见或粒细胞亚型(CML.CT)的19%到有巨核细胞增多特征患者(CML.MI)的40%不等。在进展为纤维化的速度或最终骨髓硬化率方面均未发现显著差异。在CML.MI中,大多数患者(75%)仅显示纤维增多,而在CML.CT中则伴有巨核细胞的额外增多,分别将组织学模式从CML.CT转变为.MI或.MP。因此,数据揭示了纤维增多与CML亚型之间的相关性,这正如慢性骨髓增殖性疾病的汉诺威分类法所提示的那样。有巨核细胞增多的CML亚型可显示为疾病的“骨髓纤维化前期”阶段,因此可被视为一种特殊的加速途径。

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