Orphanos V, Andonopoulos A P, Kourakli A, Zoumbos N C
Division of Hematology, Medical School, University of Patras, Greece.
Clin Exp Rheumatol. 1998 Nov-Dec;16(6):683-8.
Anemia of chronic disease is a common feature of giant cell arteritis (GCA). It has been shown that constitutive hematopoiesis is regulated in the bone marrow microenvironment by direct cell-to-cell contact between hematopoietic progenitor cells and marrow stromal cells. Since cytokines are of crucial significance in this process, we decided to examine the role of stem cell factor (SCF), granulocyte macrophage colony stimulating factor (GM-CSF), interleukin 3 (IL-3), granulocyte colony stimulating factor (G-CSF), and interleukin 1 alpha (IL-1 alpha), known for their stimulatory effects on in vitro hematopoiesis; and transforming growth factor beta (TGF beta), interferon gamma (IFN gamma) and tumor necrosis factor alpha (TNF-alpha), known for their inhibitory effect, in the anemia of GCA.
The expression of these cytokines was examined in the marrow stromal cells from 6 GCA patients with anemia of chronic disease and in 6 healthy individuals by the reverse transcription mediated polymerase chain reaction (RT-PCR).
Consistent constitutive gene expression was detected in the marrow stromal cells of all control samples for SCF, GM-CSF, IL-1 alpha, TGF beta and TNF-alpha, while only 2/6 expressed IFN gamma. None of the controls expressed either IL-3 or G-CSF. In all GCA patients SCF, TGF beta and TNF-alpha were expressed, but the level of expression was reduced compared to the control group. IL-1 alpha was expressed in 3/6 patients, and IFN gamma in 2/6. More importantly, GM-CSF expression was not detected in any patient, in marked contrast with the controls.
The combination of the defective expression of SCF and GM-CSF, cytokines with a known stimulatory effect on in vitro hematopoiesis, may contribute to the pathogenesis of anemia in GCA.
慢性病性贫血是巨细胞动脉炎(GCA)的常见特征。研究表明,组成性造血在骨髓微环境中通过造血祖细胞与骨髓基质细胞之间的直接细胞间接触来调节。由于细胞因子在这一过程中至关重要,我们决定研究干细胞因子(SCF)、粒细胞巨噬细胞集落刺激因子(GM-CSF)、白细胞介素3(IL-3)、粒细胞集落刺激因子(G-CSF)和白细胞介素1α(IL-1α)(已知对体外造血有刺激作用);以及转化生长因子β(TGFβ)、干扰素γ(IFNγ)和肿瘤坏死因子α(TNF-α)(已知有抑制作用)在GCA贫血中的作用。
通过逆转录介导的聚合酶链反应(RT-PCR)检测6例患有慢性病性贫血的GCA患者和6例健康个体骨髓基质细胞中这些细胞因子的表达。
在所有对照样本的骨髓基质细胞中均检测到SCF、GM-CSF、IL-1α、TGFβ和TNF-α的持续组成性基因表达,而只有2/6表达IFNγ。所有对照均未表达IL-3或G-CSF。在所有GCA患者中均检测到SCF、TGFβ和TNF-α表达,但与对照组相比表达水平降低。IL-1α在3/6患者中表达,IFNγ在2/6患者中表达。更重要的是,在任何患者中均未检测到GM-CSF表达,这与对照组形成明显对比。
对体外造血有已知刺激作用的细胞因子SCF和GM-CSF表达缺陷的联合作用,可能有助于GCA贫血的发病机制。