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骨髓特征的标准化——它在血液病理学中对不同疾病模式进行组织学鉴别时是否有效?

Standardization of bone marrow features--does it work in hematopathology for histological discrimination of different disease patterns?

作者信息

Thiele J, Kvasnicka H M, Diehl V

机构信息

Institute of Pathology, University of Cologne, Cologne, Germany.

出版信息

Histol Histopathol. 2005 Apr;20(2):633-44. doi: 10.14670/HH-20.633.

Abstract

Standardized bone marrow (BM) features determined by semiquantitative scoring are valuable tools for the recognition and easily reproducible interpretation of histological patterns in hematopathology. This procedure may help to characterize various disease entities, but especially to differentiate chronic myeloproliferative disorders (MPDs) with increased platelet counts from reactive thrombocytosis (RTh). A clear-cut separation of these conditions continues to present a major problem in hematology. Therefore MPDs are a most suitable model to test the diagnostic relevance of this procedure. By regarding the literature and based on archive material that involved BM biopsies of 319 patients, a semiquantitative grading of histological parameters was performed. Standardized features were applied for a stepwise discriminant analysis to establish different sets of variables exerting a diagnostic impact. A distinction into five histological patterns was achieved that showed a correctly predicted group membership of about 94 %. These were consistent with the clinicopathological diagnosis of polycythemia vera, essential thrombocythemia (ET), prefibrotic or early fibrotic chronic idiopathic myelofibrosis (CIMF) and finally RTh. Variables of discriminating potency according to their ranking included megakaryopoiesis (maturation defects, nuclear lobulation, naked and bulbous nuclei, small and giant size), reticulin fibers, erythro- and granulopoiesis (left shifting and quantity) and cellularity. These findings are in keeping with the assumption that characteristic patterns of BM histopathology can be assigned to different subtypes of MDPs mimicking ET. Discrimination between ET and especially early stage CIMF with thrombocythemia is warranted because of significant implications concerning therapeutic strategies, follow-up examinations and survival. Regarding these results, a schematic procedure is proposed to be used for daily routine diagnosis concerning the discrimination of MPDs.

摘要

通过半定量评分确定的标准化骨髓(BM)特征是血液病理学中识别组织学模式并易于重复解释的重要工具。该程序有助于表征各种疾病实体,尤其是有助于鉴别血小板计数增加的慢性骨髓增殖性疾病(MPD)与反应性血小板增多症(RTh)。在血液学中,明确区分这些情况仍然是一个主要问题。因此,MPD是测试该程序诊断相关性的最合适模型。通过查阅文献并基于涉及319例患者骨髓活检的存档材料,对组织学参数进行了半定量分级。将标准化特征应用于逐步判别分析,以建立具有诊断意义的不同变量集。实现了分为五种组织学模式的区分,其正确预测的组成员率约为94%。这些与真性红细胞增多症、原发性血小板增多症(ET)、纤维化前期或早期纤维化慢性特发性骨髓纤维化(CIMF)以及最终的RTh的临床病理诊断一致。根据其排名具有鉴别效力的变量包括巨核细胞生成(成熟缺陷、核分叶、裸核和球状核、大小不一)、网状纤维、红系和粒系生成(核左移和数量)以及细胞密度。这些发现符合以下假设,即BM组织病理学的特征模式可归因于模仿ET的MDPs的不同亚型。由于对治疗策略、随访检查和生存有重大影响,因此有必要区分ET与尤其是伴有血小板增多症的早期CIMF。基于这些结果,提出了一种用于日常常规诊断以鉴别MPD的示意性程序。

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