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骨髓的组织学和细胞遗传学特征与骨髓增生异常综合征的预后及诊断的关系

Histological and cytogenetic characterization of bone marrow in relation to prognosis and diagnosis of myelodysplastic syndromes.

作者信息

Sakuma Toshiko, Hayashi Yoshitake, Kanomata Naoki, Murayama Tohru, Matsui Toshimitsu, Kajimoto Kazuyoshi, Hanioka Keisuke, Chihara Kazuo, Maeda Sakan

机构信息

Division of Molecular Pathology, Kobe University Graduate School of Medicine, Kobe, Japan.

出版信息

Pathol Int. 2006 Apr;56(4):191-9. doi: 10.1111/j.1440-1827.2006.01945.x.

Abstract

Bone marrow (BM) histology of 102 myelodysplastic syndromes (MDS) patients was analyzed retrospectively. All the cases were reclassified according to the World Health Organization (WHO) classification. Karyotype study was conducted for all except one. Fifteen of the MDS cases were hypoplastic. The cellularity in bone marrow histology is sometimes ineffective in the differential diagnosis of MDS and aplastic anemia (AA). Nonetheless, a marked decrease in the number of megakaryocytes (average, 0.3/mm(2); range, 0-2/mm(2)) even in the hyperplastic foci of the marrow of AA was the most important histological feature differentiating AA from MDS, whereas the number of megakaryocytes increased in most MDS cases (44/mm(2); range, 1-240/mm(2)) and also in hypoplastic MDS (14/mm(2); range, 8-26/mm(2)). Hyperplastic marrow had a significantly high frequency of progress to acute myeloid leukemia (AML) and hypoplastic MDS had a lower rate of progress to AML. Severe myelofibrosis had a significantly poor prognosis. An increase in CD34-positive cells in MDS indicated a high rate of progress to AML. As for the patients with refractory cytopenia with multilineage dysplasia (RCMD; the new category under the WHO classification), the increased number of megakaryocytes was correlated with poor prognosis.

摘要

对102例骨髓增生异常综合征(MDS)患者的骨髓组织学进行了回顾性分析。所有病例均根据世界卫生组织(WHO)分类进行重新分类。除1例患者外,其余均进行了核型研究。15例MDS病例为增生低下型。骨髓组织学中的细胞计数在MDS与再生障碍性贫血(AA)的鉴别诊断中有时并无作用。尽管如此,即使在AA患者骨髓的增生灶中巨核细胞数量显著减少(平均0.3/mm²;范围0 - 2/mm²)仍是将AA与MDS区分开来的最重要组织学特征,而在大多数MDS病例中巨核细胞数量增加(44/mm²;范围1 - 240/mm²),在增生低下型MDS中也是如此(14/mm²;范围8 - 26/mm²)。增生性骨髓进展为急性髓系白血病(AML)的频率显著较高,而增生低下型MDS进展为AML的比例较低。严重骨髓纤维化的预后明显较差。MDS中CD34阳性细胞增加表明进展为AML的几率较高。对于伴有多系发育异常的难治性血细胞减少症(RCMD;WHO分类中的新类别)患者,巨核细胞数量增加与预后不良相关。

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