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骨髓增生异常综合征的预后因素

Prognostic factors in myelodysplastic syndromes.

作者信息

Triantafyllidis Irina, Ciobanu Anca, Stanca Oana, Lupu Anca Roxana

机构信息

Coltea Clinical Hospital, Department of Hematology Bucharest, Romania.

出版信息

Maedica (Bucur). 2012 Dec;7(4):295-302.

Abstract

BACKGROUND

Myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cell and are characterized by ineffective hematopoiesis with normo- or hyper cellular bone marrow and cytopenia(s).The natural evolution of the disease consists of bone marrow failure (leading to infectious and hemorrhagic episodes or anemia related complications) and transformation to acute myeloid leukemia. Because MDSs display remarkable clinical, pathologic, and cytogenetic heterogeneity, with variable evolution and survival ranging from months to years, the predictive factors of prognosis have a key role in optimal therapeutic decisions.The purpose of this paper is to analyze prognostic factors within a group of patients diagnosed with myelodysplastic syndromes. The prognostic factors taken into account are: the number and depth of cytopenias, percentage of bone marrow blasts, cytogenetic abnormalities, intensity of anemia and transfusional dependence. These factors are related to overall survival, leukemia free survival, bone marrow failure complications, leukemic evolution, treatment decisions and the response to treatment.

MATERIAL AND METHOD

The study group comprises of 119 patients diagnosed with de novo MDS, between 2008 and 2011 in the Hematology Department of Coltea Clinical Hospital. In this monitoring period the patients were stratified according to the FAB (French-American-British) morphologic classification.

RESULTS

This study revealed that the outcomes of patients with MDS is influenced by the percentage of bone marrow blasts at diagnosis, the number and severity of hematopoietic lineage affected by cytopenia and by the presence of chromosomal abnormalities.

CONCLUSIONS

The studied prognostic factors have predictive value in terms of survival, leukemic transformation, treatment response and development of bone marrow failure-related characteristic complications.

摘要

背景

骨髓增生异常综合征(MDS)是造血干细胞的克隆性疾病,其特征为造血无效伴骨髓正常或细胞增多以及血细胞减少。该疾病的自然演变包括骨髓衰竭(导致感染、出血发作或贫血相关并发症)以及转化为急性髓系白血病。由于MDS表现出显著的临床、病理和细胞遗传学异质性,其演变和生存期从数月到数年不等,因此预后的预测因素在最佳治疗决策中起着关键作用。本文旨在分析一组诊断为骨髓增生异常综合征患者的预后因素。所考虑的预后因素包括:血细胞减少的数量和程度、骨髓原始细胞百分比、细胞遗传学异常、贫血强度和输血依赖性。这些因素与总生存期、无白血病生存期、骨髓衰竭并发症、白血病演变、治疗决策以及治疗反应相关。

材料与方法

研究组包括2008年至2011年在科尔泰亚临床医院血液科诊断为原发性MDS的119例患者。在此监测期间,患者根据FAB(法美英)形态学分类进行分层。

结果

本研究表明,MDS患者的预后受诊断时骨髓原始细胞百分比、受血细胞减少影响的造血谱系数量和严重程度以及染色体异常的存在影响。

结论

所研究的预后因素在生存、白血病转化、治疗反应以及骨髓衰竭相关特征性并发症的发生方面具有预测价值。

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