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[原发性骨髓增生异常综合征的组织病理学与临床方面]

[Histopathology and clinical aspects of primary myelodysplastic syndrome].

作者信息

Maschek H, Georgii A

机构信息

Pathologisches Institut, Medizinische Hochschule Hannover.

出版信息

Pathologe. 1995 Jan;16(1):53-61. doi: 10.1007/s002920050076.

Abstract

The histopathology of bone marrow in primary myelodysplastic syndromes (MDS) is described, with reference to the FAB classification. Variants such as hypoplastic, thrombocythemic and fibrotic MDS are recognized from their histopathology and must be incorporated in the FAB classification. The clinical significance of hypoplastic, thrombocythemic and fibrotic variants is illustrated by the survival rates and leukemic transformation in these patients. Histopathological classification according to the FAB system corresponds with cytological classification, as proven by the distribution of the subtypes, blood values, leukemic transformation rates and survival times. Finally it is even possible to elaborate a prognostic score for survival based on histological features of diagnostic biopsies, which emphasizes the importance of histopathological examination of bone marrow in MDS patients.

摘要

本文参考FAB分类描述了原发性骨髓增生异常综合征(MDS)的骨髓组织病理学。从组织病理学角度识别出诸如低增生性、血小板增多性和纤维化性MDS等变体,这些变体必须纳入FAB分类。这些患者的生存率和白血病转化情况说明了低增生性、血小板增多性和纤维化性变体的临床意义。根据FAB系统进行的组织病理学分类与细胞学分类相符,亚型分布、血液指标、白血病转化率和生存时间都证明了这一点。最后,甚至可以根据诊断性活检的组织学特征制定生存预后评分,这强调了对MDS患者进行骨髓组织病理学检查的重要性。

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