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骨髓增生异常(白血病前期)综合征:骨髓造血功能衰竭问题。

Myelodysplastic (preleukemia) syndromes: the bone marrow factory failure problem.

作者信息

Hoagland H C

机构信息

Division of Hematology, Mayo Clinic Rochester, MN 55905, USA.

出版信息

Mayo Clin Proc. 1995 Jul;70(7):673-6; quiz 677. doi: 10.4065/70.7.673.

Abstract

The myelodysplastic syndromes are a group of hematologic disorders that adversely affect the levels of hemoglobin, platelets, erythrocytes, and leukocytes. Although the cause of this syndrome is unknown, new diagnostic techniques have facilitated identification and classification of these diseases into five categories: refractory anemia (refractory cytopenia), refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation, and chronic myelomonocytic leukemia. Cytogenetic abnormalities may be present in more than 55% of the patients. Symptomatic patients should be assessed relative to life-threatening versus non-life-threatening cytopenias and age. Management consists of primarily supportive measures, although certain approaches that are currently being used or under investigation, such as concomitant administration of erythropoietin and other growth factors, show promise for the future.

摘要

骨髓增生异常综合征是一组血液系统疾病,会对血红蛋白、血小板、红细胞和白细胞水平产生不利影响。尽管该综合征的病因尚不清楚,但新的诊断技术有助于对这些疾病进行识别和分类,分为五类:难治性贫血(难治性血细胞减少)、伴有环形铁粒幼细胞的难治性贫血、伴有过多原始细胞的难治性贫血、转化中的伴有过多原始细胞的难治性贫血以及慢性粒单核细胞白血病。超过55%的患者可能存在细胞遗传学异常。有症状的患者应根据危及生命与非危及生命的血细胞减少情况以及年龄进行评估。治疗主要包括支持性措施,尽管目前正在使用或正在研究的某些方法,如同时使用促红细胞生成素和其他生长因子,显示出未来的前景。

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