Sawada K
Department of Internal Medicine II, Hokkaido University School of Medicine, Sapporo, Japan.
Leuk Lymphoma. 1998 Mar;29(1-2):49-60. doi: 10.3109/10428199809058381.
Myelodysplastic syndromes (MDS) are a heterogeneous group of disorders of hematopoiesis entailing hyperproliferative and ineffective hematopoiesis associated with morphologic evidence of marrow cell dysplasia resulting in refractory cytopenia(s), and an increased risk of transformation into acute myeloblastic leukemia (AML). The administration of colony-stimulating factor(s) (CSFs) to patients with MDS increased blood neutrophil concentrations, in most patients, and it was anticipated to be of benefit to prevent infections. The progression to AML while being treated with CSFs has come under close scrutiny. In vitro studies are expected to produce more pertinent criteria for selection of patients who are likely to benefit, as well as the overall benefits of various therapies. For this purpose, in vitro colony assays are an excellent approach for investigation of the biologic characteristics of MDS progenitor cells. The stem cell phenotype CD34 is the one of the best markers of progenitor cells, and can be used for the purification of these cells to unify levels of maturation; a direct comparison of proliferative and differentiative capacity of MDS progenitor cells with normal CD34+ cells can thus be made. The properties of MDS CD34+ cells are described here in association with proliferation and differentiation, with special emphasis on the role of stem cell factor (a ligand for c-kit) in leukemic type growth of MDS CD34+ cells.
骨髓增生异常综合征(MDS)是一组异质性造血系统疾病,其特征为造血细胞过度增殖但无效,伴有骨髓细胞发育异常的形态学证据,导致难治性血细胞减少,并增加转化为急性髓细胞白血病(AML)的风险。对MDS患者使用集落刺激因子(CSF)可提高大多数患者的血液中性粒细胞浓度,预计有助于预防感染。在使用CSF治疗期间进展为AML的情况受到了密切关注。体外研究有望为选择可能受益的患者以及各种治疗的总体益处提供更相关的标准。为此,体外集落测定是研究MDS祖细胞生物学特性的一种优秀方法。干细胞表型CD34是祖细胞的最佳标志物之一,可用于纯化这些细胞以统一成熟水平;因此,可以直接比较MDS祖细胞与正常CD34+细胞的增殖和分化能力。本文描述了MDS CD34+细胞与增殖和分化相关的特性,特别强调了干细胞因子(c-kit的配体)在MDS CD34+细胞白血病样生长中的作用。